Header Logo

Robert H Brown MD, DPhil

Endowed TitleDonna M. and Robert J. Manning Chair in Neurosciences
InstitutionUMass Chan Medical School
AddressUMass Chan Medical School
55 Lake Avenue North
Worcester MA 01655
vCardDownload vCard
    Other Positions
    InstitutionT.H. Chan School of Medicine

    InstitutionT.H. Chan School of Medicine
    DepartmentNeuroNexus Institute

    InstitutionT.H. Chan School of Medicine
    DepartmentRNA Therapeutics Institute

    InstitutionMorningside Graduate School of Biomedical Sciences
    DepartmentInterdisciplinary Graduate Program

    InstitutionMorningside Graduate School of Biomedical Sciences
    DepartmentMasters in Clinical Investigation

    InstitutionMorningside Graduate School of Biomedical Sciences
    DepartmentMD/PhD Program

    InstitutionMorningside Graduate School of Biomedical Sciences

    InstitutionMorningside Graduate School of Biomedical Sciences
    DepartmentTranslational Science

    InstitutionUMass Chan Programs, Centers and Institutes
    DepartmentBiochemistry and Molecular Pharmacology

    InstitutionUMass Chan Programs, Centers and Institutes
    DepartmentBioinformatics and Integrative Biology

    Collapse Biography 
    Collapse education and training
    Amherst College, Amherst, MA, United StatesBABiophysics
    Harvard Medical School, Boston, MA, United StatesMD
    University of Oxford, Oxford, , United KingdomDPHILNeurophysiology

    Collapse Overview 
    Collapse Summary
    Dr. Brown’s laboratory has focused on the identification of gene defects that elucidate the molecular pathogenesis of selected neuromuscular diseases including amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease), muscular dystrophy, adrenoleukodystrophy, hereditary neuropathy and hyperkalemic periodic paralysis.
    Collapse overview
    Robert Brown

    Identification of Gene Defects and Neuromuscular Disease

    Dr. Brown's laboratory has focused on the identification of gene defects that elucidate the molecular pathogenesis of selected neuromuscular diseases including amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease), muscular dystrophy, adrenoleukodystrophy, hereditary neuropathy and hyperkalemic periodic paralysis. Knowledge of theses disease genes has facilitated the creation of mouse and cell-based models of these disorders. In turn, these resources have allowed study of therapeutic strategies using conventional small molecule approaches and new modalities such as inhibitory RNAi.

    Collapse Bibliographic 
    Collapse selected publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
    Newest   |   Oldest   |   Most Cited   |   Most Discussed   |   Timeline   |   Field Summary   |   Plain Text
    PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Dogan EO, Bouley J, Zhong J, Harkins AL, Keeler AM, Bosco DA, Brown RH, Henninger N. Genetic ablation of Sarm1 attenuates expression and mislocalization of phosphorylated TDP-43 after mouse repetitive traumatic brain injury. Acta Neuropathol Commun. 2023 Dec 20; 11(1):206. PMID: 38124145.
      Citations:    Fields:    Translation:AnimalsCells
    2. Kahriman A, Bouley J, Tuncali I, Dogan EO, Pereira M, Luu T, Bosco DA, Jaber S, Peters OM, Brown RH, Henninger N. Repeated mild traumatic brain injury triggers pathology in asymptomatic C9ORF72 transgenic mice. Brain. 2023 12 01; 146(12):5139-5152. PMID: 37527465.
      Citations: 1     Fields:    Translation:AnimalsCells
    3. Fang M, Deibler SK, Nana AL, Vatsavayai SC, Banday S, Zhou Y, Almeida S, Weiss A, Brown RH, Seeley WW, Gao FB, Green MR. Loss of TDP-43 function contributes to genomic instability in amyotrophic lateral sclerosis. Front Neurosci. 2023; 17:1251228. PMID: 37849894.
    4. Cabrera GT, Meijboom KE, Abdallah A, Tran H, Foster Z, Weiss A, Wightman N, Stock R, Gendron T, Gruntman A, Giampetruzzi A, Petrucelli L, Brown RH, Mueller C. Artificial microRNA suppresses C9ORF72 variants and decreases toxic dipeptide repeat proteins in vivo. Gene Ther. 2024 Mar; 31(3-4):105-118. PMID: 37752346.
      Citations: 1     Fields:    
    5. Yang H, Brown RH, Wang D, Strauss KA, Gao G. Rescue of GM3 synthase deficiency by spatially controlled, rAAV-mediated ST3GAL5 delivery. JCI Insight. 2023 05 08; 8(9). PMID: 37014712.
      Citations:    Fields:    Translation:HumansAnimals
    6. Dominov JA, Madigan LA, Whitt JP, Rademacher KL, Webster KM, Zhang H, Banno H, Tang S, Zhang Y, Wightman N, Shychuck EM, Page J, Weiss A, Kelly K, Kucukural A, Brodsky MH, Jaworski A, Fallon JR, Lipscombe D, Brown RH. Up-regulation of cholesterol synthesis pathways and limited neurodegeneration in a knock-in Sod1 mutant mouse model of ALS. bioRxiv. 2023 May 05. PMID: 37205335.
    7. Younger DS, Brown RH. Amyotrophic lateral sclerosis. Handb Clin Neurol. 2023; 196:203-229. PMID: 37620070.
      Citations:    Fields:    Translation:HumansCells
    8. Kumar MS, Fowler-Magaw ME, Kulick D, Boopathy S, Gadd DH, Rotunno M, Douthwright C, Golebiowski D, Yusuf I, Xu Z, Brown RH, Sena-Esteves M, O'Neil AL, Bosco DA. Anti-SOD1 Nanobodies That Stabilize Misfolded SOD1 Proteins Also Promote Neurite Outgrowth in Mutant SOD1 Human Neurons. Int J Mol Sci. 2022 Dec 16; 23(24). PMID: 36555655.
      Citations:    Fields:    Translation:HumansAnimalsCells
    9. Opie-Martin S, Iacoangeli A, Topp SD, Abel O, Mayl K, Mehta PR, Shatunov A, Fogh I, Bowles H, Limbachiya N, Spargo TP, Al-Khleifat A, Williams KL, Jockel-Balsarotti J, Bali T, Self W, Henden L, Nicholson GA, Ticozzi N, McKenna-Yasek D, Tang L, Shaw PJ, Chio A, Ludolph A, Weishaupt JH, Landers JE, Glass JD, Mora JS, Robberecht W, Damme PV, McLaughlin R, Hardiman O, van den Berg L, Veldink JH, Corcia P, Stevic Z, Siddique N, Silani V, Blair IP, Fan DS, Esselin F, de la Cruz E, Camu W, Basak NA, Siddique T, Miller T, Brown RH, Al-Chalabi A, Shaw CE. The SOD1-mediated ALS phenotype shows a decoupling between age of symptom onset and disease duration. Nat Commun. 2022 11 12; 13(1):6901. PMID: 36371497.
      Citations: 7     Fields:    Translation:Humans
    10. Meijboom KE, Abdallah A, Fordham NP, Nagase H, Rodriguez T, Kraus C, Gendron TF, Krishnan G, Esanov R, Andrade NS, Rybin MJ, Ramic M, Stephens ZD, Edraki A, Blackwood MT, Kahriman A, Henninger N, Kocher JA, Benatar M, Brodsky MH, Petrucelli L, Gao FB, Sontheimer EJ, Brown RH, Zeier Z, Mueller C. CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro. Nat Commun. 2022 10 21; 13(1):6286. PMID: 36271076.
      Citations: 13     Fields:    Translation:AnimalsCells
    11. Meijboom KE, Brown RH. Approaches to Gene Modulation Therapy for ALS. Neurotherapeutics. 2022 07; 19(4):1159-1179. PMID: 36068427.
      Citations: 12     Fields:    Translation:Humans
    12. Krishnan G, Raitcheva D, Bartlett D, Prudencio M, McKenna-Yasek DM, Douthwright C, Oskarsson BE, Ladha S, King OD, Barmada SJ, Miller TM, Bowser R, Watts JK, Petrucelli L, Brown RH, Kankel MW, Gao FB. Poly(GR) and poly(GA) in cerebrospinal fluid as potential biomarkers for C9ORF72-ALS/FTD. Nat Commun. 2022 05 19; 13(1):2799. PMID: 35589711.
      Citations: 15     Fields:    Translation:Humans
    13. Eitan C, Siany A, Barkan E, Olender T, van Eijk KR, Moisse M, Farhan SMK, Danino YM, Yanowski E, Marmor-Kollet H, Rivkin N, Yacovzada NS, Hung ST, Cooper-Knock J, Yu CH, Louis C, Masters SL, Kenna KP, van der Spek RAA, Sproviero W, Al Khleifat A, Iacoangeli A, Shatunov A, Jones AR, Elbaz-Alon Y, Cohen Y, Chapnik E, Rothschild D, Weissbrod O, Beck G, Ainbinder E, Ben-Dor S, Werneburg S, Schafer DP, Brown RH, Shaw PJ, Van Damme P, van den Berg LH, Phatnani H, Segal E, Ichida JK, Al-Chalabi A, Veldink JH, Hornstein E. Whole-genome sequencing reveals that variants in the Interleukin 18 Receptor Accessory Protein 3'UTR protect against ALS. Nat Neurosci. 2022 04; 25(4):433-445. PMID: 35361972.
      Citations: 12     Fields:    Translation:HumansCells
    14. Lee PJ, Kennedy Z, Wang Y, Lu Y, Cefaliello C, Uyan ?, Song CQ, Godinho BMC, Xu Z, Rusckowski M, Xue W, Brown RH. Imaging Net Retrograde Axonal Transport In Vivo: A Physiological Biomarker. Ann Neurol. 2022 05; 91(5):716-729. PMID: 35178738.
      Citations: 3     Fields:    Translation:HumansAnimals
    15. Arredondo C, Cefaliello C, Dyrda A, Jury N, Martinez P, D?az I, Amaro A, Tran H, Morales D, Pertusa M, Stoica L, Fritz E, Corval?n D, Abarz?a S, M?ndez-Ruette M, Fern?ndez P, Rojas F, Kumar MS, Aguilar R, Almeida S, Weiss A, Bustos FJ, Gonz?lez-Nilo F, Otero C, Tevy MF, Bosco DA, S?ez JC, K?hne T, Gao FB, Berry JD, Nicholson K, Sena-Esteves M, Madrid R, Varela D, Montecino M, Brown RH, van Zundert B. Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons. Neuron. 2022 05 18; 110(10):1656-1670.e12. PMID: 35276083.
      Citations: 16     Fields:    Translation:HumansAnimalsCells
    16. Thiboutot J, Yuan W, Park HC, Li D, Loube J, Mitzner W, Yarmus L, Li X, Brown RH. Visualization and Validation of The Microstructures in The Airway Wall in vivo Using Diffractive Optical Coherence Tomography. Acad Radiol. 2022 Nov; 29(11):1623-1630. PMID: 35282990.
      Citations: 2     Fields:    Translation:HumansAnimals
    17. van Rheenen W, van der Spek RAA, Bakker MK, van Vugt JJFA, Hop PJ, Zwamborn RAJ, de Klein N, Westra HJ, Bakker OB, Deelen P, Shireby G, Hannon E, Moisse M, Baird D, Restuadi R, Dolzhenko E, Dekker AM, Gawor K, Westeneng HJ, Tazelaar GHP, van Eijk KR, Kooyman M, Byrne RP, Doherty M, Heverin M, Al Khleifat A, Iacoangeli A, Shatunov A, Ticozzi N, Cooper-Knock J, Smith BN, Gromicho M, Chandran S, Pal S, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Sendtner M, Meyer T, Basak N, van der Kooi AJ, Ratti A, Fogh I, Gellera C, Lauria G, Corti S, Cereda C, Sproviero D, D'Alfonso S, Sorar? G, Siciliano G, Filosto M, Padovani A, Chi? A, Calvo A, Moglia C, Brunetti M, Canosa A, Grassano M, Beghi E, Pupillo E, Logroscino G, Nefussy B, Osmanovic A, Nordin A, Lerner Y, Zabari M, Gotkine M, Baloh RH, Bell S, Vourc'h P, Corcia P, Couratier P, Millecamps S, Meininger V, Salachas F, Mora Pardina JS, Assialioui A, Rojas-Garc?a R, Dion PA, Ross JP, Ludolph AC, Weishaupt JH, Brenner D, Freischmidt A, Bensimon G, Brice A, Durr A, Payan CAM, Saker-Delye S, Wood NW, Topp S, Rademakers R, Tittmann L, Lieb W, Franke A, Ripke S, Braun A, Kraft J, Whiteman DC, Olsen CM, Uitterlinden AG, Hofman A, Rietschel M, Cichon S, N?then MM, Amouyel P, Traynor BJ, Singleton AB, Mitne Neto M, Cauchi RJ, Ophoff RA, Wiedau-Pazos M, Lomen-Hoerth C, van Deerlin VM, Grosskreutz J, Roediger A, Gaur N, J?rk A, Barthel T, Theele E, Ilse B, Stubendorff B, Witte OW, Steinbach R, H?bner CA, Graff C, Brylev L, Fominykh V, Demeshonok V, Ataulina A, Rogelj B, Koritnik B, Zidar J, Ravnik-Glavac M, Glavac D, Stevic Z, Drory V, Povedano M, Blair IP, Kiernan MC, Benyamin B, Henderson RD, Furlong S, Mathers S, McCombe PA, Needham M, Ngo ST, Nicholson GA, Pamphlett R, Rowe DB, Steyn FJ, Williams KL, Mather KA, Sachdev PS, Henders AK, Wallace L, de Carvalho M, Pinto S, Petri S, Weber M, Rouleau GA, Silani V, Curtis CJ, Breen G, Glass JD, Brown RH, Landers JE, Shaw CE, Andersen PM, Groen EJN, van Es MA, Pasterkamp RJ, Fan D, Garton FC, McRae AF, Davey Smith G, Gaunt TR, Eberle MA, Mill J, McLaughlin RL, Hardiman O, Kenna KP, Wray NR, Tsai E, Runz H, Franke L, Al-Chalabi A, Van Damme P, van den Berg LH, Veldink JH. Author Correction: Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology. Nat Genet. 2022 Mar; 54(3):361. PMID: 35102318.
      Citations: 1     Fields:    
    18. Flotte TR, Cataltepe O, Puri A, Batista AR, Moser R, McKenna-Yasek D, Douthwright C, Gernoux G, Blackwood M, Mueller C, Tai PWL, Jiang X, Bateman S, Spanakis SG, Parzych J, Keeler AM, Abayazeed A, Rohatgi S, Gibson L, Finberg R, Barton BA, Vardar Z, Shazeeb MS, Gounis M, Tifft CJ, Eichler FS, Brown RH, Martin DR, Gray-Edwards HL, Sena-Esteves M. AAV gene therapy for Tay-Sachs disease. Nat Med. 2022 02; 28(2):251-259. PMID: 35145305.
      Citations: 28     Fields:    Translation:HumansCells
    19. Yang C, Qiao T, Yu J, Wang H, Guo Y, Salameh J, Metterville J, Parsi S, Yusuf I, Brown RH, Cai H, Xu Z. Low-level overexpression of wild type TDP-43 causes late-onset, progressive neurodegeneration and paralysis in mice. PLoS One. 2022; 17(2):e0255710. PMID: 35113871.
      Citations: 11     Fields:    
    20. Cudkowicz ME, Lindborg SR, Goyal NA, Miller RG, Burford MJ, Berry JD, Nicholson KA, Mozaffar T, Katz JS, Jenkins LJ, Baloh RH, Lewis RA, Staff NP, Owegi MA, Berry DA, Gothelf Y, Levy YS, Aricha R, Kern RZ, Windebank AJ, Brown RH. A randomized placebo-controlled phase 3 study of mesenchymal stem cells induced to secrete high levels of neurotrophic factors in amyotrophic lateral sclerosis. Muscle Nerve. 2022 03; 65(3):291-302. PMID: 34890069.
      Citations: 23     Fields:    Translation:HumansCellsCTClinical Trials
    21. Tran H, Moazami MP, Yang H, McKenna-Yasek D, Douthwright CL, Pinto C, Metterville J, Shin M, Sanil N, Dooley C, Puri A, Weiss A, Wightman N, Gray-Edwards H, Marosfoi M, King RM, Kenderdine T, Fabris D, Bowser R, Watts JK, Brown RH. Suppression of mutant C9orf72 expression by a potent mixed backbone antisense oligonucleotide. Nat Med. 2022 01; 28(1):117-124. PMID: 34949835.
      Citations: 46     Fields:    Translation:HumansAnimalsCells
    22. Bilches Medinas D, Malik S, Yildiz-B?l?kbasi E, Borgonovo J, Saaranen MJ, Urra H, Pulgar E, Afzal M, Contreras D, Wright MT, Bodaleo F, Quiroz G, Rozas P, Mumtaz S, D?az R, Rozas C, Cabral-Miranda F, Pi?a R, Valenzuela V, Uyan O, Reardon C, Woehlbier U, Brown RH, Sena-Esteves M, Gonzalez-Billault C, Morales B, Plate L, Ruddock LW, Concha ML, Hetz C, Tolun A. Mutation in protein disulfide isomerase A3 causes neurodevelopmental defects by disturbing endoplasmic reticulum proteostasis. EMBO J. 2022 12 17; 41(2):e105531. PMID: 34904718.
      Citations: 5     Fields:    Translation:HumansAnimalsCells
    23. van Rheenen W, van der Spek RAA, Bakker MK, van Vugt JJFA, Hop PJ, Zwamborn RAJ, de Klein N, Westra HJ, Bakker OB, Deelen P, Shireby G, Hannon E, Moisse M, Baird D, Restuadi R, Dolzhenko E, Dekker AM, Gawor K, Westeneng HJ, Tazelaar GHP, van Eijk KR, Kooyman M, Byrne RP, Doherty M, Heverin M, Al Khleifat A, Iacoangeli A, Shatunov A, Ticozzi N, Cooper-Knock J, Smith BN, Gromicho M, Chandran S, Pal S, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Sendtner M, Meyer T, Basak N, van der Kooi AJ, Ratti A, Fogh I, Gellera C, Lauria G, Corti S, Cereda C, Sproviero D, D'Alfonso S, Sorar? G, Siciliano G, Filosto M, Padovani A, Chi? A, Calvo A, Moglia C, Brunetti M, Canosa A, Grassano M, Beghi E, Pupillo E, Logroscino G, Nefussy B, Osmanovic A, Nordin A, Lerner Y, Zabari M, Gotkine M, Baloh RH, Bell S, Vourc'h P, Corcia P, Couratier P, Millecamps S, Meininger V, Salachas F, Mora Pardina JS, Assialioui A, Rojas-Garc?a R, Dion PA, Ross JP, Ludolph AC, Weishaupt JH, Brenner D, Freischmidt A, Bensimon G, Brice A, Durr A, Payan CAM, Saker-Delye S, Wood NW, Topp S, Rademakers R, Tittmann L, Lieb W, Franke A, Ripke S, Braun A, Kraft J, Whiteman DC, Olsen CM, Uitterlinden AG, Hofman A, Rietschel M, Cichon S, N?then MM, Amouyel P, Traynor BJ, Singleton AB, Mitne Neto M, Cauchi RJ, Ophoff RA, Wiedau-Pazos M, Lomen-Hoerth C, van Deerlin VM, Grosskreutz J, Roediger A, Gaur N, J?rk A, Barthel T, Theele E, Ilse B, Stubendorff B, Witte OW, Steinbach R, H?bner CA, Graff C, Brylev L, Fominykh V, Demeshonok V, Ataulina A, Rogelj B, Koritnik B, Zidar J, Ravnik-Glavac M, Glavac D, Stevic Z, Drory V, Povedano M, Blair IP, Kiernan MC, Benyamin B, Henderson RD, Furlong S, Mathers S, McCombe PA, Needham M, Ngo ST, Nicholson GA, Pamphlett R, Rowe DB, Steyn FJ, Williams KL, Mather KA, Sachdev PS, Henders AK, Wallace L, de Carvalho M, Pinto S, Petri S, Weber M, Rouleau GA, Silani V, Curtis CJ, Breen G, Glass JD, Brown RH, Landers JE, Shaw CE, Andersen PM, Groen EJN, van Es MA, Pasterkamp RJ, Fan D, Garton FC, McRae AF, Davey Smith G, Gaunt TR, Eberle MA, Mill J, McLaughlin RL, Hardiman O, Kenna KP, Wray NR, Tsai E, Runz H, Franke L, Al-Chalabi A, Van Damme P, van den Berg LH, Veldink JH. Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology. Nat Genet. 2021 12; 53(12):1636-1648. PMID: 34873335.
      Citations: 97     Fields:    Translation:HumansCells
    24. Johnson JO, Chia R, Miller DE, Li R, Kumaran R, Abramzon Y, Alahmady N, Renton AE, Topp SD, Gibbs JR, Cookson MR, Sabir MS, Dalgard CL, Troakes C, Jones AR, Shatunov A, Iacoangeli A, Al Khleifat A, Ticozzi N, Silani V, Gellera C, Blair IP, Dobson-Stone C, Kwok JB, Bonkowski ES, Palvadeau R, Tienari PJ, Morrison KE, Shaw PJ, Al-Chalabi A, Brown RH, Calvo A, Mora G, Al-Saif H, Gotkine M, Leigh F, Chang IJ, Perlman SJ, Glass I, Scott AI, Shaw CE, Basak AN, Landers JE, Chi? A, Crawford TO, Smith BN, Traynor BJ, Smith BN, Ticozzi N, Fallini C, Gkazi AS, Topp SD, Scotter EL, Kenna KP, Keagle P, Tiloca C, Vance C, Troakes C, Colombrita C, King A, Pensato V, Castellotti B, Baas F, Ten Asbroek ALMA, McKenna-Yasek D, McLaughlin RL, Polak M, Asress S, Esteban-P?rez J, Stevic Z, D'Alfonso S, Mazzini L, Comi GP, Del Bo R, Ceroni M, Gagliardi S, Querin G, Bertolin C, van Rheenen W, Rademakers R, van Blitterswijk M, Lauria G, Duga S, Corti S, Cereda C, Corrado L, Sorar? G, Williams KL, Nicholson GA, Blair IP, Leblond-Manry C, Rouleau GA, Hardiman O, Morrison KE, Veldink JH, van den Berg LH, Al-Chalabi A, Pall H, Shaw PJ, Turner MR, Talbot K, Taroni F, Garc?a-Redondo A, Wu Z, Glass JD, Gellera C, Ratti A, Brown RH, Silani V, Shaw CE, Landers JE, Dalgard CL, Adeleye A, Soltis AR, Alba C, Viollet C, Bacikova D, Hupalo DN, Sukumar G, Pollard HB, Wilkerson MD, Martinez EM, Abramzon Y, Ahmed S, Arepalli S, Baloh RH, Bowser R, Brady CB, Brice A, Broach J, Campbell RH, Camu W, Chia R, Cooper-Knock J, Ding J, Drepper C, Drory VE, Dunckley TL, Eicher JD, England BK, Faghri F, Feldman E, Floeter MK, Fratta P, Geiger JT, Gerhard G, Gibbs JR, Gibson SB, Glass JD, Hardy J, Harms MB, Heiman-Patterson TD, Hernandez DG, Jansson L, Kirby J, Kowall NW, Laaksovirta H, Landeck N, Landi F, Le Ber I, Lumbroso S, MacGowan DJL, Maragakis NJ, Mora G, Mouzat K, Murphy NA, Myllykangas L, Nalls MA, Orrell RW, Ostrow LW, Pamphlett R, Pickering-Brown S, Pioro EP, Pletnikova O, Pliner HA, Pulst SM, Ravits JM, Renton AE, Rivera A, Robberecht W, Rogaeva E, Rollinson S, Rothstein JD, Scholz SW, Sendtner M, Shaw PJ, Sidle KC, Simmons Z, Singleton AB, Smith N, Stone DJ, Tienari PJ, Troncoso JC, Valori M, Van Damme P, Van Deerlin VM, Van Den Bosch L, Zinman L, Landers JE, Chi? A, Traynor BJ, Angelocola SM, Ausiello FP, Barberis M, Bartolomei I, Battistini S, Bersano E, Bisogni G, Borghero G, Brunetti M, Cabona C, Calvo A, Canale F, Canosa A, Cantisani TA, Capasso M, Caponnetto C, Cardinali P, Carrera P, Casale F, Chi? A, Colletti T, Conforti FL, Conte A, Conti E, Corbo M, Cuccu S, Dalla Bella E, D'Errico E, DeMarco G, Dubbioso R, Ferrarese C, Ferraro PM, Filippi M, Fini N, Floris G, Fuda G, Gallone S, Gianferrari G, Giannini F, Grassano M, Greco L, Iazzolino B, Introna A, La Bella V, Lattante S, Lauria G, Liguori R, Logroscino G, Logullo FO, Lunetta C, Mandich P, Mandrioli J, Manera U, Manganelli F, Marangi G, Marinou K, Marrosu MG, Martinelli I, Messina S, Moglia C, Mora G, Mosca L, Murru MR, Origone P, Passaniti C, Petrelli C, Petrucci A, Pozzi S, Pugliatti M, Quattrini A, Ricci C, Riolo G, Riva N, Russo M, Sabatelli M, Salamone P, Salivetto M, Salvi F, Santarelli M, Sbaiz L, Sideri R, Simone I, Simonini C, Spataro R, Tanel R, Tedeschi G, Ticca A, Torriello A, Tranquilli S, Tremolizzo L, Trojsi F, Vasta R, Vacchiano V, Vita G, Volanti P, Zollino M, Zucchi E. Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis. JAMA Neurol. 2021 10 01; 78(10):1236-1248. PMID: 34459874.
      Citations: 31     Fields:    Translation:Humans
    25. Mohassel P, Donkervoort S, Lone MA, Nalls M, Gable K, Gupta SD, Foley AR, Hu Y, Saute JAM, Moreira AL, Kok F, Introna A, Logroscino G, Grunseich C, Nickolls AR, Pourshafie N, Neuhaus SB, Saade D, Gangfu? A, K?lbel H, Piccus Z, Le Pichon CE, Fiorillo C, Ly CV, T?pf A, Brady L, Specht S, Zidell A, Pedro H, Mittelmann E, Thomas FP, Chao KR, Konersman CG, Cho MT, Brandt T, Straub V, Connolly AM, Schara U, Roos A, Tarnopolsky M, H?ke A, Brown RH, Lee CH, Hornemann T, Dunn TM, B?nnemann CG. Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis. Nat Med. 2021 Jul; 27(7):1197-1204. PMID: 34059824.
      Citations: 49     Fields:    Translation:HumansCells
    26. Peters OM, Weiss A, Metterville J, Song L, Logan R, Smith GA, Schwarzschild MA, Mueller C, Brown RH, Freeman M. Genetic diversity of axon degenerative mechanisms in models of Parkinson's disease. Neurobiol Dis. 2021 07; 155:105368. PMID: 33892050.
      Citations: 7     Fields:    Translation:AnimalsCells
    27. Quezada E, Cappelli C, Diaz I, Jury N, Wightman N, Brown RH, Montecino M, van Zundert B. BET bromodomain inhibitors PFI-1 and JQ1 are identified in an epigenetic compound screen to enhance C9ORF72 gene expression and shown to ameliorate C9ORF72-associated pathological and behavioral abnormalities in a C9ALS/FTD model. Clin Epigenetics. 2021 03 16; 13(1):56. PMID: 33726839.
      Citations: 4     Fields:    Translation:HumansAnimalsCells
    28. Yang H, Brown RH, Wang D, Strauss KA, Gao G. AAV-Mediated Gene Therapy for Glycosphingolipid Biosynthesis Deficiencies. Trends Mol Med. 2021 06; 27(6):520-523. PMID: 33714697.
      Citations: 5     Fields:    Translation:HumansCells
    29. Liu Y, Dodart JC, Tran H, Berkovitch S, Braun M, Byrne M, Durbin AF, Hu XS, Iwamoto N, Jang HG, Kandasamy P, Liu F, Longo K, Ruschel J, Shelke J, Yang H, Yin Y, Donner A, Zhong Z, Vargeese C, Brown RH. Variant-selective stereopure oligonucleotides protect against pathologies associated with C9orf72-repeat expansion in preclinical models. Nat Commun. 2021 02 08; 12(1):847. PMID: 33558503.
      Citations: 32     Fields:    Translation:AnimalsCells
    30. Quinn C, Mcmillan CT, Owegi MA, Almasy K, Douthwright C, Mckenna-Yasek D, Goyal NA, Berry J, Brown RH. Single breath counting is an effective screening tool for forced vital capacity in ALS. Amyotroph Lateral Scler Frontotemporal Degener. 2021; 22(sup1):5-8. PMID: 34348533.
      Citations:    Fields:    Translation:HumansCellsPHPublic Health
    31. Wainger BJ, Brown RH. Amyotrophic Lateral Sclerosis: Fuel for the Corticofugal Feud. Ann Neurol. 2020 10; 88(4):682-684. PMID: 32741003.
      Citations: 2     Fields:    Translation:Humans
    32. Mueller C, Berry JD, McKenna-Yasek DM, Gernoux G, Owegi MA, Pothier LM, Douthwright CL, Gelevski D, Luppino SD, Blackwood M, Wightman NS, Oakley DH, Frosch MP, Flotte TR, Cudkowicz ME, Brown RH. SOD1 Suppression with Adeno-Associated Virus and MicroRNA in Familial ALS. N Engl J Med. 2020 07 09; 383(2):151-158. PMID: 32640133.
      Citations: 85     Fields:    Translation:HumansCells
    33. Huang F, Zhu Y, Hsiao-Nakamoto J, Tang X, Dugas JC, Moscovitch-Lopatin M, Glass JD, Brown RH, Ladha SS, Lacomis D, Harris JM, Scearce-Levie K, Ho C, Bowser R, Berry JD. Longitudinal biomarkers in amyotrophic lateral sclerosis. Ann Clin Transl Neurol. 2020 07; 7(7):1103-1116. PMID: 32515902.
      Citations: 34     Fields:    Translation:Humans
    34. Keeler AM, Zieger M, Semple C, Pucci L, Veinbachs A, Brown RH, Mueller C, ElMallah MK. Intralingual and Intrapleural AAV Gene Therapy Prolongs Survival in a SOD1 ALS Mouse Model. Mol Ther Methods Clin Dev. 2020 Jun 12; 17:246-257. PMID: 31970202.
    35. Reichenstein I, Eitan C, Diaz-Garcia S, Haim G, Magen I, Siany A, Hoye ML, Rivkin N, Olender T, Toth B, Ravid R, Mandelbaum AD, Yanowski E, Liang J, Rymer JK, Levy R, Beck G, Ainbinder E, Farhan SMK, Lennox KA, Bode NM, Behlke MA, M?ller T, Saxena S, Moreno CAM, Costaguta G, van Eijk KR, Phatnani H, Al-Chalabi A, Basak AN, van den Berg LH, Hardiman O, Landers JE, Mora JS, Morrison KE, Shaw PJ, Veldink JH, Pfaff SL, Yizhar O, Gross C, Brown RH, Ravits JM, Harms MB, Miller TM, Hornstein E. Human genetics and neuropathology suggest a link between miR-218 and amyotrophic lateral sclerosis pathophysiology. Sci Transl Med. 2019 12 18; 11(523). PMID: 31852800.
      Citations: 26     Fields:    Translation:HumansAnimalsCells
    36. Berry JD, Cudkowicz ME, Windebank AJ, Staff NP, Owegi M, Nicholson K, McKenna-Yasek D, Levy YS, Abramov N, Kaspi H, Mehra M, Aricha R, Gothelf Y, Brown RH. NurOwn, phase 2, randomized, clinical trial in patients with ALS: Safety, clinical, and biomarker results. Neurology. 2019 12 10; 93(24):e2294-e2305. PMID: 31740545.
      Citations: 49     Fields:    Translation:HumansCTClinical Trials
    37. Cammack AJ, Atassi N, Hyman T, van den Berg LH, Harms M, Baloh RH, Brown RH, van Es MA, Veldink JH, de Vries BS, Rothstein JD, Drain C, Jockel-Balsarotti J, Malcolm A, Boodram S, Salter A, Wightman N, Yu H, Sherman AV, Esparza TJ, McKenna-Yasek D, Owegi MA, Douthwright C, McCampbell A, Ferguson T, Cruchaga C, Cudkowicz M, Miller TM. Prospective natural history study of C9orf72 ALS clinical characteristics and biomarkers. Neurology. 2019 10 22; 93(17):e1605-e1617. PMID: 31578300.
      Citations: 14     Fields:    Translation:HumansCells
    38. Giampetruzzi A, Danielson EW, Gumina V, Jeon M, Boopathy S, Brown RH, Ratti A, Landers JE, Fallini C. Modulation of actin polymerization affects nucleocytoplasmic transport in multiple forms of amyotrophic lateral sclerosis. Nat Commun. 2019 08 23; 10(1):3827. PMID: 31444357.
      Citations: 36     Fields:    Translation:HumansCells
    39. Gelfman S, Dugger S, de Araujo Martins Moreno C, Ren Z, Wolock CJ, Shneider NA, Phatnani H, Cirulli ET, Lasseigne BN, Harris T, Maniatis T, Rouleau GA, Brown RH, Gitler AD, Myers RM, Petrovski S, Allen A, Goldstein DB, Harms MB. A new approach for rare variation collapsing on functional protein domains implicates specific genic regions in ALS. Genome Res. 2019 05; 29(5):809-818. PMID: 30940688.
      Citations: 13     Fields:    Translation:HumansCells
    40. Watts JK, Brown RH, Khvorova A. Nucleic Acid Therapeutics for Neurological Diseases. Neurotherapeutics. 2019 04; 16(2):245-247. PMID: 31037650.
      Citations: 11     Fields:    Translation:HumansAnimals
    41. Dominov JA, Uyan ?, McKenna-Yasek D, Nallamilli BRR, Kergourlay V, Bartoli M, Levy N, Hudson J, Evangelista T, Lochmuller H, Krahn M, Rufibach L, Hegde M, Brown RH. Correction of pseudoexon splicing caused by a novel intronic dysferlin mutation. Ann Clin Transl Neurol. 2019 Apr; 6(4):642-654. PMID: 31019989.
      Citations: 11     Fields:    Translation:HumansCells
    42. Peters OM, Lewis EA, Osterloh JM, Weiss A, Salameh JS, Metterville J, Brown RH, Freeman MR. Loss of Sarm1 does not suppress motor neuron degeneration in the SOD1G93A mouse model of amyotrophic lateral sclerosis. Hum Mol Genet. 2018 11 01; 27(21):3761-3771. PMID: 30010873.
      Citations: 27     Fields:    Translation:AnimalsCells
    43. Borel F, Gernoux G, Sun H, Stock R, Blackwood M, Brown RH, Mueller C. Safe and effective superoxide dismutase 1 silencing using artificial microRNA in macaques. Sci Transl Med. 2018 10 31; 10(465). PMID: 30381409.
      Citations: 35     Fields:    Translation:AnimalsCells
    44. Bouley J, Chung DY, Ayata C, Brown RH, Henninger N. Cortical Spreading Depression Denotes Concussion Injury. J Neurotrauma. 2019 04 01; 36(7):1008-1017. PMID: 29999455.
      Citations: 18     Fields:    Translation:Animals
    45. White MA, Kim E, Duffy A, Adalbert R, Phillips BU, Peters OM, Stephenson J, Yang S, Massenzio F, Lin Z, Andrews S, Segonds-Pichon A, Metterville J, Saksida LM, Mead R, Ribchester RR, Barhomi Y, Serre T, Coleman MP, Fallon JR, Bussey TJ, Brown RH, Sreedharan J. Publisher Correction: TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD. Nat Neurosci. 2018 Aug; 21(8):1138. PMID: 29872124.
      Citations: 2     Fields:    
    46. Medinas DB, Rozas P, Mart?nez Traub F, Woehlbier U, Brown RH, Bosco DA, Hetz C. Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2018 08 07; 115(32):8209-8214. PMID: 30038021.
      Citations: 52     Fields:    Translation:HumansAnimalsCells
    47. Braun MC, Castillo-Ruiz A, Indic P, Jung DY, Kim JK, Brown RH, Swoap SJ, Schwartz WJ. Defective daily temperature regulation in a mouse model of amyotrophic lateral sclerosis. Exp Neurol. 2019 01; 311:305-312. PMID: 30031021.
      Citations: 1     Fields:    Translation:HumansAnimals
    48. de Majo M, Topp SD, Smith BN, Nishimura AL, Chen HJ, Gkazi AS, Miller J, Wong CH, Vance C, Baas F, Ten Asbroek ALMA, Kenna KP, Ticozzi N, Redondo AG, Esteban-P?rez J, Tiloca C, Verde F, Duga S, Morrison KE, Shaw PJ, Kirby J, Turner MR, Talbot K, Hardiman O, Glass JD, de Belleroche J, Gellera C, Ratti A, Al-Chalabi A, Brown RH, Silani V, Landers JE, Shaw CE. ALS-associated missense and nonsense TBK1 mutations can both cause loss of kinase function. Neurobiol Aging. 2018 11; 71:266.e1-266.e10. PMID: 30033073.
      Citations: 35     Fields:    Translation:HumansCells
    49. Ghasemi M, Brown RH. Genetics of Amyotrophic Lateral Sclerosis. Cold Spring Harb Perspect Med. 2018 05 01; 8(5). PMID: 28270533.
      Citations: 75     Fields:    Translation:Humans
    50. Al-Chalabi A, Brown RH. Finding a Treatment for ALS - Will Gene Editing Cut It? N Engl J Med. 2018 Apr 12; 378(15):1454-1456. PMID: 29641956.
      Citations: 6     Fields:    Translation:HumansAnimalsCells
    51. Nicolas A, Kenna KP, Renton AE, Ticozzi N, Faghri F, Chia R, Dominov JA, Kenna BJ, Nalls MA, Keagle P, Rivera AM, van Rheenen W, Murphy NA, van Vugt JJFA, Geiger JT, Van der Spek RA, Pliner HA, Smith BN, Marangi G, Topp SD, Abramzon Y, Gkazi AS, Eicher JD, Kenna A, Mora G, Calvo A, Mazzini L, Riva N, Mandrioli J, Caponnetto C, Battistini S, Volanti P, La Bella V, Conforti FL, Borghero G, Messina S, Simone IL, Trojsi F, Salvi F, Logullo FO, D'Alfonso S, Corrado L, Capasso M, Ferrucci L, Moreno CAM, Kamalakaran S, Goldstein DB, Gitler AD, Harris T, Myers RM, Phatnani H, Musunuri RL, Evani US, Abhyankar A, Zody MC, Kaye J, Finkbeiner S, Wyman SK, LeNail A, Lima L, Fraenkel E, Svendsen CN, Thompson LM, Van Eyk JE, Berry JD, Miller TM, Kolb SJ, Cudkowicz M, Baxi E, Benatar M, Taylor JP, Rampersaud E, Wu G, Wuu J, Lauria G, Verde F, Fogh I, Tiloca C, Comi GP, Sorar? G, Cereda C, Corcia P, Laaksovirta H, Myllykangas L, Jansson L, Valori M, Ealing J, Hamdalla H, Rollinson S, Pickering-Brown S, Orrell RW, Sidle KC, Malaspina A, Hardy J, Singleton AB, Johnson JO, Arepalli S, Sapp PC, McKenna-Yasek D, Polak M, Asress S, Al-Sarraj S, King A, Troakes C, Vance C, de Belleroche J, Baas F, Ten Asbroek ALMA, Mu?oz-Blanco JL, Hernandez DG, Ding J, Gibbs JR, Scholz SW, Floeter MK, Campbell RH, Landi F, Bowser R, Pulst SM, Ravits JM, MacGowan DJL, Kirby J, Pioro EP, Pamphlett R, Broach J, Gerhard G, Dunckley TL, Brady CB, Kowall NW, Troncoso JC, Le Ber I, Mouzat K, Lumbroso S, Heiman-Patterson TD, Kamel F, Van Den Bosch L, Baloh RH, Strom TM, Meitinger T, Shatunov A, Van Eijk KR, de Carvalho M, Kooyman M, Middelkoop B, Moisse M, McLaughlin RL, Van Es MA, Weber M, Boylan KB, Van Blitterswijk M, Rademakers R, Morrison KE, Basak AN, Mora JS, Drory VE, Shaw PJ, Turner MR, Talbot K, Hardiman O, Williams KL, Fifita JA, Nicholson GA, Blair IP, Rouleau GA, Esteban-P?rez J, Garc?a-Redondo A, Al-Chalabi A, Rogaeva E, Zinman L, Ostrow LW, Maragakis NJ, Rothstein JD, Simmons Z, Cooper-Knock J, Brice A, Goutman SA, Feldman EL, Gibson SB, Taroni F, Ratti A, Gellera C, Van Damme P, Robberecht W, Fratta P, Sabatelli M, Lunetta C, Ludolph AC, Andersen PM, Weishaupt JH, Camu W, Trojanowski JQ, Van Deerlin VM, Brown RH, van den Berg LH, Veldink JH, Harms MB, Glass JD, Stone DJ, Tienari P, Silani V, Chi? A, Shaw CE, Traynor BJ, Landers JE. Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron. 2018 03 21; 97(6):1267-1288. PMID: 29566793.
      Citations: 304     Fields:    Translation:HumansCells
    52. White MA, Kim E, Duffy A, Adalbert R, Phillips BU, Peters OM, Stephenson J, Yang S, Massenzio F, Lin Z, Andrews S, Segonds-Pichon A, Metterville J, Saksida LM, Mead R, Ribchester RR, Barhomi Y, Serre T, Coleman MP, Fallon JR, Bussey TJ, Brown RH, Sreedharan J. TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD. Nat Neurosci. 2018 04; 21(4):552-563. PMID: 29556029.
      Citations: 103     Fields:    Translation:AnimalsCells
    53. Fournier CN, Schoenfeld D, Berry JD, Cudkowicz ME, Chan J, Quinn C, Brown RH, Salameh JS, Tansey MG, Beers DR, Appel SH, Glass JD. An open label study of a novel immunosuppression intervention for the treatment of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2018 05; 19(3-4):242-249. PMID: 29308669.
      Citations: 15     Fields:    Translation:Humans
    54. Brown RH, Al-Chalabi A. Amyotrophic Lateral Sclerosis. N Engl J Med. 2017 07 13; 377(2):162-172. PMID: 28700839.
      Citations: 609     Fields:    Translation:Humans
    55. Esanov R, Cabrera GT, Andrade NS, Gendron TF, Brown RH, Benatar M, Wahlestedt C, Mueller C, Zeier Z. A C9ORF72 BAC mouse model recapitulates key epigenetic perturbations of ALS/FTD. Mol Neurodegener. 2017 06 12; 12(1):46. PMID: 28606110.
      Citations: 16     Fields:    Translation:AnimalsCells
    56. Smith BN, Topp SD, Fallini C, Shibata H, Chen HJ, Troakes C, King A, Ticozzi N, Kenna KP, Soragia-Gkazi A, Miller JW, Sato A, Dias DM, Jeon M, Vance C, Wong CH, de Majo M, Kattuah W, Mitchell JC, Scotter EL, Parkin NW, Sapp PC, Nolan M, Nestor PJ, Simpson M, Weale M, Lek M, Baas F, Vianney de Jong JM, Ten Asbroek ALMA, Redondo AG, Esteban-P?rez J, Tiloca C, Verde F, Duga S, Leigh N, Pall H, Morrison KE, Al-Chalabi A, Shaw PJ, Kirby J, Turner MR, Talbot K, Hardiman O, Glass JD, De Belleroche J, Maki M, Moss SE, Miller C, Gellera C, Ratti A, Al-Sarraj S, Brown RH, Silani V, Landers JE, Shaw CE. Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis. Sci Transl Med. 2017 05 03; 9(388). PMID: 28469040.
      Citations: 60     Fields:    Translation:HumansCells
    57. Gendron TF, Chew J, Stankowski JN, Hayes LR, Zhang YJ, Prudencio M, Carlomagno Y, Daughrity LM, Jansen-West K, Perkerson EA, O'Raw A, Cook C, Pregent L, Belzil V, van Blitterswijk M, Tabassian LJ, Lee CW, Yue M, Tong J, Song Y, Castanedes-Casey M, Rousseau L, Phillips V, Dickson DW, Rademakers R, Fryer JD, Rush BK, Pedraza O, Caputo AM, Desaro P, Palmucci C, Robertson A, Heckman MG, Diehl NN, Wiggs E, Tierney M, Braun L, Farren J, Lacomis D, Ladha S, Fournier CN, McCluskey LF, Elman LB, Toledo JB, McBride JD, Tiloca C, Morelli C, Poletti B, Solca F, Prelle A, Wuu J, Jockel-Balsarotti J, Rigo F, Ambrose C, Datta A, Yang W, Raitcheva D, Antognetti G, McCampbell A, Van Swieten JC, Miller BL, Boxer AL, Brown RH, Bowser R, Miller TM, Trojanowski JQ, Grossman M, Berry JD, Hu WT, Ratti A, Traynor BJ, Disney MD, Benatar M, Silani V, Glass JD, Floeter MK, Rothstein JD, Boylan KB, Petrucelli L. Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis. Sci Transl Med. 2017 03 29; 9(383). PMID: 28356511.
      Citations: 118     Fields:    Translation:HumansAnimalsCells
    58. Brown RH, Schwartz WJ. David A. Drachman, MD (1932-2016). Neurology. 2017 03 07; 88(10):928-929. PMID: 28265038.
      Citations:    Fields:    
    59. Stoica L, Keeler AM, Xiong L, Kalfopoulos M, Desrochers K, Brown RH, Sena-Esteves M, Flotte TR, ElMallah MK. Restrictive Lung Disease in the Cu/Zn Superoxide-Dismutase 1 G93A Amyotrophic Lateral Sclerosis Mouse Model. Am J Respir Cell Mol Biol. 2017 03; 56(3):405-408. PMID: 28248134.
      Citations: 7     Fields:    Translation:HumansAnimals
    60. Fil D, DeLoach A, Yadav S, Alkam D, MacNicol M, Singh A, Compadre CM, Goellner JJ, O'Brien CA, Fahmi T, Basnakian AG, Calingasan NY, Klessner JL, Beal FM, Peters OM, Metterville J, Brown RH, Ling KKY, Rigo F, Ozdinler PH, Kiaei M. Mutant Profilin1 transgenic mice recapitulate cardinal features of motor neuron disease. Hum Mol Genet. 2017 02 15; 26(4):686-701. PMID: 28040732.
      Citations: 45     Fields:    Translation:HumansAnimals
    61. Czell D, Sapp PC, Neuwirth C, Weber M, Andersen PM, Brown RH. Further analysis of KIFAP3 gene in ALS patients from Switzerland and Sweden. Amyotroph Lateral Scler Frontotemporal Degener. 2017 05; 18(3-4):302-304. PMID: 28140676.
      Citations: 2     Fields:    Translation:Humans
    62. Taylor JP, Brown RH, Cleveland DW. Decoding ALS: from genes to mechanism. Nature. 2016 Nov 10; 539(7628):197-206. PMID: 27830784.
      Citations: 881     Fields:    Translation:HumansAnimalsCells
    63. Yang C, Danielson EW, Qiao T, Metterville J, Brown RH, Landers JE, Xu Z. Mutant PFN1 causes ALS phenotypes and progressive motor neuron degeneration in mice by a gain of toxicity. Proc Natl Acad Sci U S A. 2016 10 11; 113(41):E6209-E6218. PMID: 27681617.
      Citations: 41     Fields:    Translation:HumansAnimalsCells
    64. van Zundert B, Brown RH. Silencing strategies for therapy of SOD1-mediated ALS. Neurosci Lett. 2017 01 01; 636:32-39. PMID: 27507699.
      Citations: 34     Fields:    Translation:HumansAnimals
    65. van Rheenen W, Shatunov A, Dekker AM, McLaughlin RL, Diekstra FP, Pulit SL, van der Spek RA, V?sa U, de Jong S, Robinson MR, Yang J, Fogh I, van Doormaal PT, Tazelaar GH, Koppers M, Blokhuis AM, Sproviero W, Jones AR, Kenna KP, van Eijk KR, Harschnitz O, Schellevis RD, Brands WJ, Medic J, Menelaou A, Vajda A, Ticozzi N, Lin K, Rogelj B, Vrabec K, Ravnik-Glavac M, Koritnik B, Zidar J, Leonardis L, Gro?elj LD, Millecamps S, Salachas F, Meininger V, de Carvalho M, Pinto S, Mora JS, Rojas-Garc?a R, Polak M, Chandran S, Colville S, Swingler R, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Pittman A, Sidle K, Fratta P, Malaspina A, Topp S, Petri S, Abdulla S, Drepper C, Sendtner M, Meyer T, Ophoff RA, Staats KA, Wiedau-Pazos M, Lomen-Hoerth C, Van Deerlin VM, Trojanowski JQ, Elman L, McCluskey L, Basak AN, Tunca C, Hamzeiy H, Parman Y, Meitinger T, Lichtner P, Radivojkov-Blagojevic M, Andres CR, Maurel C, Bensimon G, Landwehrmeyer B, Brice A, Payan CA, Saker-Delye S, D?rr A, Wood NW, Tittmann L, Lieb W, Franke A, Rietschel M, Cichon S, N?then MM, Amouyel P, Tzourio C, Dartigues JF, Uitterlinden AG, Rivadeneira F, Estrada K, Hofman A, Curtis C, Blauw HM, van der Kooi AJ, de Visser M, Goris A, Weber M, Shaw CE, Smith BN, Pansarasa O, Cereda C, Del Bo R, Comi GP, D'Alfonso S, Bertolin C, Sorar? G, Mazzini L, Pensato V, Gellera C, Tiloca C, Ratti A, Calvo A, Moglia C, Brunetti M, Arcuti S, Capozzo R, Zecca C, Lunetta C, Penco S, Riva N, Padovani A, Filosto M, Muller B, Stuit RJ, Blair I, Zhang K, McCann EP, Fifita JA, Nicholson GA, Rowe DB, Pamphlett R, Kiernan MC, Grosskreutz J, Witte OW, Ringer T, Prell T, Stubendorff B, Kurth I, H?bner CA, Leigh PN, Casale F, Chio A, Beghi E, Pupillo E, Tortelli R, Logroscino G, Powell J, Ludolph AC, Weishaupt JH, Robberecht W, Van Damme P, Franke L, Pers TH, Brown RH, Glass JD, Landers JE, Hardiman O, Andersen PM, Corcia P, Vourc'h P, Silani V, Wray NR, Visscher PM, de Bakker PI, van Es MA, Pasterkamp RJ, Lewis CM, Breen G, Al-Chalabi A, van den Berg LH, Veldink JH. Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis. Nat Genet. 2016 09; 48(9):1043-8. PMID: 27455348.
      Citations: 262     Fields:    Translation:Humans
    66. Kenna KP, van Doormaal PT, Dekker AM, Ticozzi N, Kenna BJ, Diekstra FP, van Rheenen W, van Eijk KR, Jones AR, Keagle P, Shatunov A, Sproviero W, Smith BN, van Es MA, Topp SD, Kenna A, Miller JW, Fallini C, Tiloca C, McLaughlin RL, Vance C, Troakes C, Colombrita C, Mora G, Calvo A, Verde F, Al-Sarraj S, King A, Calini D, de Belleroche J, Baas F, van der Kooi AJ, de Visser M, Ten Asbroek AL, Sapp PC, McKenna-Yasek D, Polak M, Asress S, Mu?oz-Blanco JL, Strom TM, Meitinger T, Morrison KE, Lauria G, Williams KL, Leigh PN, Nicholson GA, Blair IP, Leblond CS, Dion PA, Rouleau GA, Pall H, Shaw PJ, Turner MR, Talbot K, Taroni F, Boylan KB, Van Blitterswijk M, Rademakers R, Esteban-P?rez J, Garc?a-Redondo A, Van Damme P, Robberecht W, Chio A, Gellera C, Drepper C, Sendtner M, Ratti A, Glass JD, Mora JS, Basak NA, Hardiman O, Ludolph AC, Andersen PM, Weishaupt JH, Brown RH, Al-Chalabi A, Silani V, Shaw CE, van den Berg LH, Veldink JH, Landers JE. NEK1 variants confer susceptibility to amyotrophic lateral sclerosis. Nat Genet. 2016 09; 48(9):1037-42. PMID: 27455347.
      Citations: 113     Fields:    Translation:Humans
    67. Fogh I, Lin K, Tiloca C, Rooney J, Gellera C, Diekstra FP, Ratti A, Shatunov A, van Es MA, Proitsi P, Jones A, Sproviero W, Chi? A, McLaughlin RL, Sorar? G, Corrado L, Stahl D, Del Bo R, Cereda C, Castellotti B, Glass JD, Newhouse S, Dobson R, Smith BN, Topp S, van Rheenen W, Meininger V, Melki J, Morrison KE, Shaw PJ, Leigh PN, Andersen PM, Comi GP, Ticozzi N, Mazzini L, D'Alfonso S, Traynor BJ, Van Damme P, Robberecht W, Brown RH, Landers JE, Hardiman O, Lewis CM, van den Berg LH, Shaw CE, Veldink JH, Silani V, Al-Chalabi A, Powell J. Association of a Locus in the CAMTA1 Gene With Survival in Patients With Sporadic Amyotrophic Lateral Sclerosis. JAMA Neurol. 2016 07 01; 73(7):812-20. PMID: 27244217.
      Citations: 29     Fields:    Translation:Humans
    68. Williams KL, Topp S, Yang S, Smith B, Fifita JA, Warraich ST, Zhang KY, Farrawell N, Vance C, Hu X, Chesi A, Leblond CS, Lee A, Rayner SL, Sundaramoorthy V, Dobson-Stone C, Molloy MP, van Blitterswijk M, Dickson DW, Petersen RC, Graff-Radford NR, Boeve BF, Murray ME, Pottier C, Don E, Winnick C, McCann EP, Hogan A, Daoud H, Levert A, Dion PA, Mitsui J, Ishiura H, Takahashi Y, Goto J, Kost J, Gellera C, Gkazi AS, Miller J, Stockton J, Brooks WS, Boundy K, Polak M, Mu?oz-Blanco JL, Esteban-P?rez J, R?bano A, Hardiman O, Morrison KE, Ticozzi N, Silani V, de Belleroche J, Glass JD, Kwok JB, Guillemin GJ, Chung RS, Tsuji S, Brown RH, Garc?a-Redondo A, Rademakers R, Landers JE, Gitler AD, Rouleau GA, Cole NJ, Yerbury JJ, Atkin JD, Shaw CE, Nicholson GA, Blair IP. CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia. Nat Commun. 2016 Apr 15; 7:11253. PMID: 27080313.
      Citations: 92     Fields:    Translation:HumansAnimalsCells
    69. Stoica L, Todeasa SH, Cabrera GT, Salameh JS, ElMallah MK, Mueller C, Brown RH, Sena-Esteves M. Adeno-associated virus-delivered artificial microRNA extends survival and delays paralysis in an amyotrophic lateral sclerosis mouse model. Ann Neurol. 2016 Apr; 79(4):687-700. PMID: 26891182.
      Citations: 50     Fields:    Translation:AnimalsCells
    70. Weiss MD, Macklin EA, Simmons Z, Knox AS, Greenblatt DJ, Atassi N, Graves M, Parziale N, Salameh JS, Quinn C, Brown RH, Distad JB, Trivedi J, Shefner JM, Barohn RJ, Pestronk A, Swenson A, Cudkowicz ME. A randomized trial of mexiletine in ALS: Safety and effects on muscle cramps and progression. Neurology. 2016 Apr 19; 86(16):1474-81. PMID: 26911633.
      Citations: 29     Fields:    Translation:HumansCTClinical Trials
    71. Woehlbier U, Colombo A, Saaranen MJ, P?rez V, Ojeda J, Bustos FJ, Andreu CI, Torres M, Valenzuela V, Medinas DB, Rozas P, Vidal RL, Lopez-Gonzalez R, Salameh J, Fernandez-Collemann S, Mu?oz N, Matus S, Armisen R, Sagredo A, Palma K, Irrazabal T, Almeida S, Gonzalez-Perez P, Campero M, Gao FB, Henny P, van Zundert B, Ruddock LW, Concha ML, Henriquez JP, Brown RH, Hetz C. ALS-linked protein disulfide isomerase variants cause motor dysfunction. EMBO J. 2016 Apr 15; 35(8):845-65. PMID: 26869642.
      Citations: 43     Fields:    Translation:HumansAnimalsCells
    72. Henninger N, Bouley J, Sikoglu EM, An J, Moore CM, King JA, Bowser R, Freeman MR, Brown RH. Attenuated traumatic axonal injury and improved functional outcome after traumatic brain injury in mice lacking Sarm1. Brain. 2016 Apr; 139(Pt 4):1094-105. PMID: 26912636.
      Citations: 99     Fields:    Translation:AnimalsCells
    73. Borel F, Gernoux G, Cardozo B, Metterville JP, Toro Cabrera GC, Song L, Su Q, Gao GP, Elmallah MK, Brown RH, Mueller C. Therapeutic rAAVrh10 Mediated SOD1 Silencing in Adult SOD1(G93A) Mice and Nonhuman Primates. Hum Gene Ther. 2016 01; 27(1):19-31. PMID: 26710998.
      Citations: 49     Fields:    Translation:HumansAnimalsCells
    74. Peters OM, Cabrera GT, Tran H, Gendron TF, McKeon JE, Metterville J, Weiss A, Wightman N, Salameh J, Kim J, Sun H, Boylan KB, Dickson D, Kennedy Z, Lin Z, Zhang YJ, Daughrity L, Jung C, Gao FB, Sapp PC, Horvitz HR, Bosco DA, Brown SP, de Jong P, Petrucelli L, Mueller C, Brown RH. Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice. Neuron. 2015 Dec 02; 88(5):902-909. PMID: 26637797.
      Citations: 150     Fields:    Translation:HumansAnimalsCells
    75. Salameh JS, Brown RH, Berry JD. Amyotrophic Lateral Sclerosis: Review. Semin Neurol. 2015 Aug; 35(4):469-76. PMID: 26502769.
      Citations: 30     Fields:    Translation:Humans
    76. Brown RH, Al-Chalabi A. Endogenous retroviruses in ALS: A reawakening? Sci Transl Med. 2015 Sep 30; 7(307):307fs40. PMID: 26424566.
      Citations: 6     Fields:    Translation:HumansAnimalsCells
    77. Sreedharan J, Neukomm LJ, Brown RH, Freeman MR. Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2. Curr Biol. 2015 Aug 17; 25(16):2130-6. PMID: 26234214.
      Citations: 34     Fields:    Translation:HumansAnimalsCells
    78. Dominov JA, Uyan ?, Sapp PC, McKenna-Yasek D, Nallamilli BR, Hegde M, Brown RH. Reply to comment on: A novel dysferlin mutant pseudoexon bypassed with antisense oligonucleotides. Ann Clin Transl Neurol. 2015 Jul; 2(7):785. PMID: 26290895.
      Citations:    Fields:    
    79. Peters OM, Ghasemi M, Brown RH. Emerging mechanisms of molecular pathology in ALS. J Clin Invest. 2015 May; 125(5):1767-79. PMID: 25932674.
      Citations: 116     Fields:    Translation:HumansAnimalsCells
    80. Gonzalez-Perez P, Woehlbier U, Chian RJ, Sapp P, Rouleau GA, Leblond CS, Daoud H, Dion PA, Landers JE, Hetz C, Brown RH. Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients. Gene. 2015 Jul 25; 566(2):158-65. PMID: 25913742.
      Citations: 32     Fields:    Translation:HumansCells
    81. Cheever TR, Berkley D, Braun S, Brown RH, Byrne BJ, Chamberlain JS, Cwik V, Duan D, Federoff HJ, High KA, Kaspar BK, Klinger KW, Larkindale J, Lincecum J, Mavilio F, McDonald CL, McLaughlin J, Weiss McLeod B, Mendell JR, Nuckolls G, Stedman HH, Tagle DA, Vandenberghe LH, Wang H, Wernett PJ, Wilson JM, Porter JD, Gubitz AK. Perspectives on best practices for gene therapy programs. Hum Gene Ther. 2015 Mar; 26(3):127-33. PMID: 25654329.
      Citations: 7     Fields:    Translation:Humans
    82. Fridman V, Oaklander AL, David WS, Johnson EA, Pan J, Novak P, Brown RH, Eichler FS. Natural history and biomarkers in hereditary sensory neuropathy type 1. Muscle Nerve. 2015 Apr; 51(4):489-95. PMID: 25042817.
      Citations: 16     Fields:    Translation:Humans
    83. ?zoguz A, Uyan ?, Birdal G, Iskender C, Kartal E, Lahut S, ?m?r ?, Agim ZS, Eken AG, Sen NE, Kavak P, Saygi C, Sapp PC, Keagle P, Parman Y, Tan E, Ko? F, Deymeer F, Oflazer P, Hanagasi H, G?rvit H, Bilgi? B, Durmus H, Ertas M, Kotan D, Akalin MA, G?ll?oglu H, Zarifoglu M, Aysal F, D?soglu N, Bilguvar K, G?nel M, Keskin ?, Akg?n T, ?z?elik H, Landers JE, Brown RH, Basak AN. The distinct genetic pattern of ALS in Turkey and novel mutations. Neurobiol Aging. 2015 Apr; 36(4):1764.e9-1764.e18. PMID: 25681989.
      Citations: 33     Fields:    Translation:Humans
    84. Smith BN, Vance C, Scotter EL, Troakes C, Wong CH, Topp S, Maekawa S, King A, Mitchell JC, Lund K, Al-Chalabi A, Ticozzi N, Silani V, Sapp P, Brown RH, Landers JE, Al-Sarraj S, Shaw CE. Novel mutations support a role for Profilin 1 in the pathogenesis of ALS. Neurobiol Aging. 2015 Mar; 36(3):1602.e17-27. PMID: 25499087.
      Citations: 50     Fields:    Translation:HumansAnimalsCells
    85. Smith BN, Ticozzi N, Fallini C, Gkazi AS, Topp S, Kenna KP, Scotter EL, Kost J, Keagle P, Miller JW, Calini D, Vance C, Danielson EW, Troakes C, Tiloca C, Al-Sarraj S, Lewis EA, King A, Colombrita C, Pensato V, Castellotti B, de Belleroche J, Baas F, ten Asbroek AL, Sapp PC, McKenna-Yasek D, McLaughlin RL, Polak M, Asress S, Esteban-P?rez J, Mu?oz-Blanco JL, Simpson M, van Rheenen W, Diekstra FP, Lauria G, Duga S, Corti S, Cereda C, Corrado L, Sorar? G, Morrison KE, Williams KL, Nicholson GA, Blair IP, Dion PA, Leblond CS, Rouleau GA, Hardiman O, Veldink JH, van den Berg LH, Al-Chalabi A, Pall H, Shaw PJ, Turner MR, Talbot K, Taroni F, Garc?a-Redondo A, Wu Z, Glass JD, Gellera C, Ratti A, Brown RH, Silani V, Shaw CE, Landers JE. Exome-wide rare variant analysis identifies TUBA4A mutations associated with familial ALS. Neuron. 2014 Oct 22; 84(2):324-31. PMID: 25374358.
      Citations: 168     Fields:    Translation:HumansCells
    86. Dominov JA, Uyan O, Sapp PC, McKenna-Yasek D, Nallamilli BR, Hegde M, Brown RH. A novel dysferlin mutant pseudoexon bypassed with antisense oligonucleotides. Ann Clin Transl Neurol. 2014 Sep; 1(9):703-20. PMID: 25493284.
      Citations: 15     Fields:    
    87. Su Z, Zhang Y, Gendron TF, Bauer PO, Chew J, Yang WY, Fostvedt E, Jansen-West K, Belzil VV, Desaro P, Johnston A, Overstreet K, Oh SY, Todd PK, Berry JD, Cudkowicz ME, Boeve BF, Dickson D, Floeter MK, Traynor BJ, Morelli C, Ratti A, Silani V, Rademakers R, Brown RH, Rothstein JD, Boylan KB, Petrucelli L, Disney MD. Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS. Neuron. 2014 Sep 03; 83(5):1043-50. PMID: 25132468.
      Citations: 177     Fields:    Translation:HumansAnimalsCells
    88. Brown RH. Obesity and asthma: "What we've got here is failure to communicate". Am J Respir Crit Care Med. 2014 Jun 15; 189(12):i-ii. PMID: 24930541.
      Citations: 1     Fields:    Translation:Humans
    89. Kiskinis E, Sandoe J, Williams LA, Boulting GL, Moccia R, Wainger BJ, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle FT, Davis-Dusenbery BN, Ziller M, Oakley D, Ichida J, Di Costanzo S, Atwater N, Maeder ML, Goodwin MJ, Nemesh J, Handsaker RE, Paull D, Noggle S, McCarroll SA, Joung JK, Woolf CJ, Brown RH, Eggan K. Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell. 2014 Jun 05; 14(6):781-95. PMID: 24704492.
      Citations: 229     Fields:    Translation:HumansCells
    90. Yang C, Wang H, Qiao T, Yang B, Aliaga L, Qiu L, Tan W, Salameh J, McKenna-Yasek DM, Smith T, Peng L, Moore MJ, Brown RH, Cai H, Xu Z. Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2014 Mar 25; 111(12):E1121-9. PMID: 24616503.
      Citations: 94     Fields:    Translation:Animals
    91. Seijffers R, Zhang J, Matthews JC, Chen A, Tamrazian E, Babaniyi O, Selig M, Hynynen M, Woolf CJ, Brown RH. ATF3 expression improves motor function in the ALS mouse model by promoting motor neuron survival and retaining muscle innervation. Proc Natl Acad Sci U S A. 2014 Jan 28; 111(4):1622-7. PMID: 24474789.
      Citations: 33     Fields:    Translation:AnimalsCells
    92. Goris A, van Setten J, Diekstra F, Ripke S, Patsopoulos NA, Sawcer SJ, van Es M, Andersen PM, Melki J, Meininger V, Hardiman O, Landers JE, Brown RH, Shatunov A, Leigh N, Al-Chalabi A, Shaw CE, Traynor BJ, Chi? A, Restagno G, Mora G, Ophoff RA, Oksenberg JR, Van Damme P, Compston A, Robberecht W, Dubois B, van den Berg LH, De Jager PL, Veldink JH, de Bakker PI. No evidence for shared genetic basis of common variants in multiple sclerosis and amyotrophic lateral sclerosis. Hum Mol Genet. 2014 Apr 01; 23(7):1916-22. PMID: 24234648.
      Citations: 13     Fields:    Translation:Humans
    93. Takahashi Y, Fukuda Y, Yoshimura J, Toyoda A, Kurppa K, Moritoyo H, Belzil VV, Dion PA, Higasa K, Doi K, Ishiura H, Mitsui J, Date H, Ahsan B, Matsukawa T, Ichikawa Y, Moritoyo T, Ikoma M, Hashimoto T, Kimura F, Murayama S, Onodera O, Nishizawa M, Yoshida M, Atsuta N, Sobue G, Fifita JA, Williams KL, Blair IP, Nicholson GA, Gonzalez-Perez P, Brown RH, Nomoto M, Elenius K, Rouleau GA, Fujiyama A, Morishita S, Goto J, Tsuji S. ERBB4 mutations that disrupt the neuregulin-ErbB4 pathway cause amyotrophic lateral sclerosis type 19. Am J Hum Genet. 2013 Nov 07; 93(5):900-5. PMID: 24119685.
      Citations: 63     Fields:    Translation:HumansCells
    94. Wang H, Yang B, Qiu L, Yang C, Kramer J, Su Q, Guo Y, Brown RH, Gao G, Xu Z. Widespread spinal cord transduction by intrathecal injection of rAAV delivers efficacious RNAi therapy for amyotrophic lateral sclerosis. Hum Mol Genet. 2014 Feb 01; 23(3):668-81. PMID: 24108104.
      Citations: 55     Fields:    Translation:HumansAnimalsCells
    95. Sreedharan J, Brown RH. Amyotrophic lateral sclerosis: Problems and prospects. Ann Neurol. 2013 Sep; 74(3):309-16. PMID: 24038380.
      Citations: 64     Fields:    Translation:HumansCells
    96. Auclair JR, Johnson JL, Liu Q, Salisbury JP, Rotunno MS, Petsko GA, Ringe D, Brown RH, Bosco DA, Agar JN. Post-translational modification by cysteine protects Cu/Zn-superoxide dismutase from oxidative damage. Biochemistry. 2013 Sep 10; 52(36):6137-44. PMID: 23927036.
      Citations: 18     Fields:    Translation:HumansAnimalsCells
    97. Morfini GA, Bosco DA, Brown H, Gatto R, Kaminska A, Song Y, Molla L, Baker L, Marangoni MN, Berth S, Tavassoli E, Bagnato C, Tiwari A, Hayward LJ, Pigino GF, Watterson DM, Huang CF, Banker G, Brown RH, Brady ST. Inhibition of fast axonal transport by pathogenic SOD1 involves activation of p38 MAP kinase. PLoS One. 2013; 8(6):e65235. PMID: 23776455.
      Citations: 62     Fields:    Translation:AnimalsCells
    98. Fritz E, Izaurieta P, Weiss A, Mir FR, Rojas P, Gonzalez D, Rojas F, Brown RH, Madrid R, van Zundert B. Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability. J Neurophysiol. 2013 Jun; 109(11):2803-14. PMID: 23486205.
      Citations: 49     Fields:    Translation:HumansAnimalsCells
    99. Peng L, Liu H, Ruan H, Tepp WH, Stoothoff WH, Brown RH, Johnson EA, Yao WD, Zhang SC, Dong M. Cytotoxicity of botulinum neurotoxins reveals a direct role of syntaxin 1 and SNAP-25 in neuron survival. Nat Commun. 2013; 4:1472. PMID: 23403573.
      Citations: 38     Fields:    Translation:HumansAnimalsCells
    100. Li S, Sheng J, Hu JK, Yu W, Kishikawa H, Hu MG, Shima K, Wu D, Xu Z, Xin W, Sims KB, Landers JE, Brown RH, Hu GF. Ribonuclease 4 protects neuron degeneration by promoting angiogenesis, neurogenesis, and neuronal survival under stress. Angiogenesis. 2013 Apr; 16(2):387-404. PMID: 23143660.
      Citations: 39     Fields:    Translation:HumansAnimalsCells
    101. Ahmeti KB, Ajroud-Driss S, Al-Chalabi A, Andersen PM, Armstrong J, Birve A, Blauw HM, Brown RH, Bruijn L, Chen W, Chio A, Comeau MC, Cronin S, Diekstra FP, Soraya Gkazi A, Glass JD, Grab JD, Groen EJ, Haines JL, Hardiman O, Heller S, Huang J, Hung WY, Jaworski JM, Jones A, Khan H, Landers JE, Langefeld CD, Leigh PN, Marion MC, McLaughlin RL, Meininger V, Melki J, Miller JW, Mora G, Pericak-Vance MA, Rampersaud E, Robberecht W, Russell LP, Salachas F, Saris CG, Shatunov A, Shaw CE, Siddique N, Siddique T, Smith BN, Sufit R, Topp S, Traynor BJ, Vance C, van Damme P, van den Berg LH, van Es MA, van Vught PW, Veldink JH, Yang Y, Zheng JG. Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1. Neurobiol Aging. 2013 Jan; 34(1):357.e7-19. PMID: 22959728.
      Citations: 47     Fields:    Translation:HumansCells
    102. Wu CH, Fallini C, Ticozzi N, Keagle PJ, Sapp PC, Piotrowska K, Lowe P, Koppers M, McKenna-Yasek D, Baron DM, Kost JE, Gonzalez-Perez P, Fox AD, Adams J, Taroni F, Tiloca C, Leclerc AL, Chafe SC, Mangroo D, Moore MJ, Zitzewitz JA, Xu ZS, van den Berg LH, Glass JD, Siciliano G, Cirulli ET, Goldstein DB, Salachas F, Meininger V, Rossoll W, Ratti A, Gellera C, Bosco DA, Bassell GJ, Silani V, Drory VE, Brown RH, Landers JE. Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature. 2012 Aug 23; 488(7412):499-503. PMID: 22801503.
      Citations: 288     Fields:    Translation:HumansAnimalsCells
    103. Gonz?lez-P?rez P, Lu Y, Chian RJ, Sapp PC, Tanzi RE, Bertram L, McKenna-Yasek D, Gao FB, Brown RH. Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS. Neurobiol Dis. 2012 Dec; 48(3):391-8. PMID: 22766032.
      Citations: 10     Fields:    Translation:Humans
    104. Osterloh JM, Yang J, Rooney TM, Fox AN, Adalbert R, Powell EH, Sheehan AE, Avery MA, Hackett R, Logan MA, MacDonald JM, Ziegenfuss JS, Milde S, Hou YJ, Nathan C, Ding A, Brown RH, Conforti L, Coleman M, Tessier-Lavigne M, Z?chner S, Freeman MR. dSarm/Sarm1 is required for activation of an injury-induced axon death pathway. Science. 2012 Jul 27; 337(6093):481-4. PMID: 22678360.
      Citations: 333     Fields:    Translation:AnimalsCells
    105. Wright PD, Wightman N, Huang M, Weiss A, Sapp PC, Cuny GD, Ivinson AJ, Glicksman MA, Ferrante RJ, Matson W, Matson S, Brown RH. A high-throughput screen to identify inhibitors of SOD1 transcription. Front Biosci (Elite Ed). 2012 06 01; 4(8):2701-8. PMID: 22652679.
      Citations: 8     Fields:    Translation:HumansAnimalsCells
    106. Diekstra FP, Saris CG, van Rheenen W, Franke L, Jansen RC, van Es MA, van Vught PW, Blauw HM, Groen EJ, Horvath S, Estrada K, Rivadeneira F, Hofman A, Uitterlinden AG, Robberecht W, Andersen PM, Melki J, Meininger V, Hardiman O, Landers JE, Brown RH, Shatunov A, Shaw CE, Leigh PN, Al-Chalabi A, Ophoff RA, van den Berg LH, Veldink JH. Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. PLoS One. 2012; 7(4):e35333. PMID: 22509407.
      Citations: 31     Fields:    Translation:HumansCells
    107. Garofalo K, Penno A, Schmidt BP, Lee HJ, Frosch MP, von Eckardstein A, Brown RH, Hornemann T, Eichler FS. Oral L-serine supplementation reduces production of neurotoxic deoxysphingolipids in mice and humans with hereditary sensory autonomic neuropathy type 1. J Clin Invest. 2011 Dec; 121(12):4735-45. PMID: 22045570.
      Citations: 98     Fields:    Translation:HumansAnimalsCTClinical Trials
    108. van Blitterswijk M, Gulati S, Smoot E, Jaffa M, Maher N, Hyman BT, Ivinson AJ, Scherzer CR, Schoenfeld DA, Cudkowicz ME, Brown RH, Bosco DA. Anti-superoxide dismutase antibodies are associated with survival in patients with sporadic amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2011 Nov; 12(6):430-8. PMID: 22023190.
      Citations: 15     Fields:    Translation:HumansAnimals
    109. Duff RM, Tay V, Hackman P, Ravenscroft G, McLean C, Kennedy P, Steinbach A, Sch?ffler W, van der Ven PFM, F?rst DO, Song J, Djinovic-Carugo K, Penttil? S, Raheem O, Reardon K, Malandrini A, Gambelli S, Villanova M, Nowak KJ, Williams DR, Landers JE, Brown RH, Udd B, Laing NG. Mutations in the N-terminal actin-binding domain of filamin C cause a distal myopathy. Am J Hum Genet. 2011 Jun 10; 88(6):729-740. PMID: 21620354.
      Citations: 54     Fields:    Translation:HumansCells
    110. Ju S, Tardiff DF, Han H, Divya K, Zhong Q, Maquat LE, Bosco DA, Hayward LJ, Brown RH, Lindquist S, Ringe D, Petsko GA. A yeast model of FUS/TLS-dependent cytotoxicity. PLoS Biol. 2011 Apr; 9(4):e1001052. PMID: 21541368.
      Citations: 127     Fields:    Translation:AnimalsCells
    111. Ozdinler PH, Benn S, Yamamoto TH, G?zel M, Brown RH, Macklis JD. Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G??A transgenic ALS mice. J Neurosci. 2011 Mar 16; 31(11):4166-77. PMID: 21411657.
      Citations: 100     Fields:    Translation:AnimalsCells
    112. Bosco DA, Morfini G, Karabacak NM, Song Y, Gros-Louis F, Pasinelli P, Goolsby H, Fontaine BA, Lemay N, McKenna-Yasek D, Frosch MP, Agar JN, Julien JP, Brady ST, Brown RH. Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci. 2010 Nov; 13(11):1396-403. PMID: 20953194.
      Citations: 338     Fields:    Translation:HumansCells
    113. Royce-Nagel G, Cudkowicz M, Myers D, Nicholson K, Shui A, Schoenfeld D, Huang X, Brown RH. Vanadium, aluminum, magnesium and manganese are not elevated in hair samples in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010 Oct; 11(5):492-3. PMID: 20438306.
      Citations: 2     Fields:    Translation:HumansPHPublic Health
    114. Shatunov A, Mok K, Newhouse S, Weale ME, Smith B, Vance C, Johnson L, Veldink JH, van Es MA, van den Berg LH, Robberecht W, Van Damme P, Hardiman O, Farmer AE, Lewis CM, Butler AW, Abel O, Andersen PM, Fogh I, Silani V, Chi? A, Traynor BJ, Melki J, Meininger V, Landers JE, McGuffin P, Glass JD, Pall H, Leigh PN, Hardy J, Brown RH, Powell JF, Orrell RW, Morrison KE, Shaw PJ, Shaw CE, Al-Chalabi A. Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurol. 2010 Oct; 9(10):986-94. PMID: 20801717.
      Citations: 106     Fields:    Translation:HumansCells
    115. Bosco DA, Lemay N, Ko HK, Zhou H, Burke C, Kwiatkowski TJ, Sapp P, McKenna-Yasek D, Brown RH, Hayward LJ. Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules. Hum Mol Genet. 2010 Nov 01; 19(21):4160-75. PMID: 20699327.
      Citations: 274     Fields:    Translation:HumansAnimalsCells
    116. Blauw HM, Al-Chalabi A, Andersen PM, van Vught PW, Diekstra FP, van Es MA, Saris CG, Groen EJ, van Rheenen W, Koppers M, Van't Slot R, Strengman E, Estrada K, Rivadeneira F, Hofman A, Uitterlinden AG, Kiemeney LA, Vermeulen SH, Birve A, Waibel S, Meyer T, Cronin S, McLaughlin RL, Hardiman O, Sapp PC, Tobin MD, Wain LV, Tomik B, Slowik A, Lemmens R, Rujescu D, Schulte C, Gasser T, Brown RH, Landers JE, Robberecht W, Ludolph AC, Ophoff RA, Veldink JH, van den Berg LH. A large genome scan for rare CNVs in amyotrophic lateral sclerosis. Hum Mol Genet. 2010 Oct 15; 19(20):4091-9. PMID: 20685689.
      Citations: 28     Fields:    Translation:HumansCells
    117. Ticozzi N, LeClerc AL, Keagle PJ, Glass JD, Wills AM, van Blitterswijk M, Bosco DA, Rodriguez-Leyva I, Gellera C, Ratti A, Taroni F, McKenna-Yasek D, Sapp PC, Silani V, Furlong CE, Brown RH, Landers JE. Paraoxonase gene mutations in amyotrophic lateral sclerosis. Ann Neurol. 2010 Jul; 68(1):102-7. PMID: 20582942.
      Citations: 29     Fields:    Translation:HumansCells
    118. Huang EJ, Zhang J, Geser F, Trojanowski JQ, Strober JB, Dickson DW, Brown RH, Shapiro BE, Lomen-Hoerth C. Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions. Brain Pathol. 2010 Nov; 20(6):1069-76. PMID: 20579074.
      Citations: 74     Fields:    Translation:HumansCells
    119. Pedrini S, Sau D, Guareschi S, Bogush M, Brown RH, Naniche N, Kia A, Trotti D, Pasinelli P. ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2. Hum Mol Genet. 2010 Aug 01; 19(15):2974-86. PMID: 20460269.
      Citations: 74     Fields:    Translation:HumansAnimalsCells
    120. Lemmens R, Moore MJ, Al-Chalabi A, Brown RH, Robberecht W. RNA metabolism and the pathogenesis of motor neuron diseases. Trends Neurosci. 2010 May; 33(5):249-58. PMID: 20227117.
      Citations: 40     Fields:    Translation:HumansAnimalsCells
    121. Glover LE, Newton K, Krishnan G, Bronson R, Boyle A, Krivickas LS, Brown RH. Dysferlin overexpression in skeletal muscle produces a progressive myopathy. Ann Neurol. 2010 Mar; 67(3):384-93. PMID: 20373350.
      Citations: 21     Fields:    Translation:HumansAnimalsCells
    122. Penno A, Reilly MM, Houlden H, Laur? M, Rentsch K, Niederkofler V, Stoeckli ET, Nicholson G, Eichler F, Brown RH, von Eckardstein A, Hornemann T. Hereditary sensory neuropathy type 1 is caused by the accumulation of two neurotoxic sphingolipids. J Biol Chem. 2010 Apr 09; 285(15):11178-87. PMID: 20097765.
      Citations: 158     Fields:    Translation:HumansAnimalsCells
    123. Ryan DP, da Silva MR, Soong TW, Fontaine B, Donaldson MR, Kung AW, Jongjaroenprasert W, Liang MC, Khoo DH, Cheah JS, Ho SC, Bernstein HS, Maciel RM, Brown RH, Pt?cek LJ. Mutations in potassium channel Kir2.6 cause susceptibility to thyrotoxic hypokalemic periodic paralysis. Cell. 2010 Jan 08; 140(1):88-98. PMID: 20074522.
      Citations: 110     Fields:    Translation:HumansCells
    124. Brown RH. Medicine. A reinnervating microRNA. Science. 2009 Dec 11; 326(5959):1494-5. PMID: 20007892.
      Citations: 9     Fields:    Translation:AnimalsCells
    125. Wain LV, Pedroso I, Landers JE, Breen G, Shaw CE, Leigh PN, Brown RH, Tobin MD, Al-Chalabi A. The role of copy number variation in susceptibility to amyotrophic lateral sclerosis: genome-wide association study and comparison with published loci. PLoS One. 2009 Dec 04; 4(12):e8175. PMID: 19997636.
      Citations: 22     Fields:    Translation:HumansCells
    126. Li J, Chian RJ, Ay I, Kashi BB, Celia SA, Tamrazian E, Pepinsky RB, Fishman PS, Brown RH, Francis JW. Insect GDNF:TTC fusion protein improves delivery of GDNF to mouse CNS. Biochem Biophys Res Commun. 2009 Dec 18; 390(3):947-51. PMID: 19852934.
      Citations: 8     Fields:    Translation:AnimalsCells
    127. Morfini GA, Burns M, Binder LI, Kanaan NM, LaPointe N, Bosco DA, Brown RH, Brown H, Tiwari A, Hayward L, Edgar J, Nave KA, Garberrn J, Atagi Y, Song Y, Pigino G, Brady ST. Axonal transport defects in neurodegenerative diseases. J Neurosci. 2009 Oct 14; 29(41):12776-86. PMID: 19828789.
      Citations: 247     Fields:    Translation:HumansAnimalsCells
    128. Hetz C, Thielen P, Matus S, Nassif M, Court F, Kiffin R, Martinez G, Cuervo AM, Brown RH, Glimcher LH. XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. Genes Dev. 2009 Oct 01; 23(19):2294-306. PMID: 19762508.
      Citations: 279     Fields:    Translation:HumansAnimalsCells
    129. van Es MA, Veldink JH, Saris CG, Blauw HM, van Vught PW, Birve A, Lemmens R, Schelhaas HJ, Groen EJ, Huisman MH, van der Kooi AJ, de Visser M, Dahlberg C, Estrada K, Rivadeneira F, Hofman A, Zwarts MJ, van Doormaal PT, Rujescu D, Strengman E, Giegling I, Muglia P, Tomik B, Slowik A, Uitterlinden AG, Hendrich C, Waibel S, Meyer T, Ludolph AC, Glass JD, Purcell S, Cichon S, N?then MM, Wichmann HE, Schreiber S, Vermeulen SH, Kiemeney LA, Wokke JH, Cronin S, McLaughlin RL, Hardiman O, Fumoto K, Pasterkamp RJ, Meininger V, Melki J, Leigh PN, Shaw CE, Landers JE, Al-Chalabi A, Brown RH, Robberecht W, Andersen PM, Ophoff RA, van den Berg LH. Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet. 2009 Oct; 41(10):1083-7. PMID: 19734901.
      Citations: 178     Fields:    Translation:HumansCells
    130. Broom WJ, Johnson DV, Garber M, Andersen PM, Lennon N, Landers J, Nusbaum C, Russ C, Brown RH. DNA sequence analysis of the conserved region around the SOD1 gene locus in recessively inherited ALS. Neurosci Lett. 2009 Sep 29; 463(1):64-9. PMID: 19596401.
      Citations: 2     Fields:    Translation:HumansCells
    131. Li J, Chian RJ, Ay I, Celia SA, Kashi BB, Tamrazian E, Matthews JC, Remington MP, Pepinsky RB, Fishman PS, Brown RH, Francis JW. Recombinant GDNF: tetanus toxin fragment C fusion protein produced from insect cells. Biochem Biophys Res Commun. 2009 Jul 31; 385(3):380-4. PMID: 19465006.
      Citations: 1     Fields:    Translation:AnimalsCells
    132. Landers JE, Melki J, Meininger V, Glass JD, van den Berg LH, van Es MA, Sapp PC, van Vught PW, McKenna-Yasek DM, Blauw HM, Cho TJ, Polak M, Shi L, Wills AM, Broom WJ, Ticozzi N, Silani V, Ozoguz A, Rodriguez-Leyva I, Veldink JH, Ivinson AJ, Saris CG, Hosler BA, Barnes-Nessa A, Couture N, Wokke JH, Kwiatkowski TJ, Ophoff RA, Cronin S, Hardiman O, Diekstra FP, Leigh PN, Shaw CE, Simpson CL, Hansen VK, Powell JF, Corcia P, Salachas F, Heath S, Galan P, Georges F, Horvitz HR, Lathrop M, Purcell S, Al-Chalabi A, Brown RH. Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2009 Jun 02; 106(22):9004-9. PMID: 19451621.
      Citations: 85     Fields:    Translation:HumansCells
    133. Han G, Gupta SD, Gable K, Niranjanakumari S, Moitra P, Eichler F, Brown RH, Harmon JM, Dunn TM. Identification of small subunits of mammalian serine palmitoyltransferase that confer distinct acyl-CoA substrate specificities. Proc Natl Acad Sci U S A. 2009 May 19; 106(20):8186-91. PMID: 19416851.
      Citations: 127     Fields:    Translation:HumansAnimalsCells
    134. Brown RH, Wagner EM, Cope KA, Risby TH. Propofol and in vivo oxidative stress: effects of preservative. J Breath Res. 2009 Mar; 3(1):016003. PMID: 21383451.
      Citations: 3     Fields:    
    135. Chiu IM, Chen A, Zheng Y, Kosaras B, Tsiftsoglou SA, Vartanian TK, Brown RH, Carroll MC. T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS. Proc Natl Acad Sci U S A. 2008 Nov 18; 105(46):17913-8. PMID: 18997009.
      Citations: 186     Fields:    Translation:HumansAnimalsCells
    136. Simpson CL, Lemmens R, Miskiewicz K, Broom WJ, Hansen VK, van Vught PW, Landers JE, Sapp P, Van Den Bosch L, Knight J, Neale BM, Turner MR, Veldink JH, Ophoff RA, Tripathi VB, Beleza A, Shah MN, Proitsi P, Van Hoecke A, Carmeliet P, Horvitz HR, Leigh PN, Shaw CE, van den Berg LH, Sham PC, Powell JF, Verstreken P, Brown RH, Robberecht W, Al-Chalabi A. Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Hum Mol Genet. 2009 Feb 01; 18(3):472-81. PMID: 18996918.
      Citations: 124     Fields:    Translation:HumansAnimalsCells
    137. van Zundert B, Peuscher MH, Hynynen M, Chen A, Neve RL, Brown RH, Constantine-Paton M, Bellingham MC. Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis. J Neurosci. 2008 Oct 22; 28(43):10864-74. PMID: 18945894.
      Citations: 125     Fields:    Translation:HumansAnimalsCells
    138. Landers JE, Shi L, Cho TJ, Glass JD, Shaw CE, Leigh PN, Diekstra F, Polak M, Rodriguez-Leyva I, Niemann S, Traynor BJ, McKenna-Yasek D, Sapp PC, Al-Chalabi A, Wills AM, Brown RH. A common haplotype within the PON1 promoter region is associated with sporadic ALS. Amyotroph Lateral Scler. 2008 Oct; 9(5):306-14. PMID: 18618303.
      Citations: 20     Fields:    Translation:HumansCells
    139. Qureshi M, Brown RH, Rogers JT, Cudkowicz ME. Serum ferritin and metal levels as risk factors for amyotrophic lateral sclerosis. Open Neurol J. 2008 Sep 12; 2:51-4. PMID: 19452011.
    140. Brown RH. Developmental biology. Neuron research leaps ahead. Science. 2008 Aug 29; 321(5893):1169-70. PMID: 18755965.
      Citations:    Fields:    Translation:HumansAnimalsCells
    141. Broom WJ, Greenway M, Sadri-Vakili G, Russ C, Auwarter KE, Glajch KE, Dupre N, Swingler RJ, Purcell S, Hayward C, Sapp PC, McKenna-Yasek D, Valdmanis PN, Bouchard JP, Meininger V, Hosler BA, Glass JD, Polack M, Rouleau GA, Cha JH, Hardiman O, Brown RH. 50bp deletion in the promoter for superoxide dismutase 1 (SOD1) reduces SOD1 expression in vitro and may correlate with increased age of onset of sporadic amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008 Aug; 9(4):229-37. PMID: 18608091.
      Citations: 12     Fields:    Translation:HumansCells
    142. Cudkowicz ME, Shefner JM, Simpson E, Grasso D, Yu H, Zhang H, Shui A, Schoenfeld D, Brown RH, Wieland S, Barber JR. Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis. Muscle Nerve. 2008 Jul; 38(1):837-44. PMID: 18551622.
      Citations: 43     Fields:    Translation:Humans
    143. Meisler MH, Russ C, Montgomery KT, Greenway M, Ennis S, Hardiman O, Figlewicz DA, Quenneville NR, Conibear E, Brown RH. Evaluation of the Golgi trafficking protein VPS54 (wobbler) as a candidate for ALS. Amyotroph Lateral Scler. 2008 Jun; 9(3):141-8. PMID: 18574757.
      Citations: 15     Fields:    Translation:HumansCells
    144. Brown RH, Kaczka DW, Fallano K, Chen S, Mitzner W. Temporal variability in the responses of individual canine airways to methacholine. J Appl Physiol (1985). 2008 May; 104(5):1381-6. PMID: 18309091.
      Citations: 7     Fields:    Translation:Animals
    145. Mills C, Makwana M, Wallace A, Benn S, Schmidt H, Tegeder I, Costigan M, Brown RH, Raivich G, Woolf CJ. Ro5-4864 promotes neonatal motor neuron survival and nerve regeneration in adult rats. Eur J Neurosci. 2008 Feb; 27(4):937-46. PMID: 18333964.
      Citations: 20     Fields:    Translation:HumansAnimalsCells
    146. Vivekananda U, Johnston C, McKenna-Yasek D, Shaw CE, Leigh PN, Brown RH, Al-Chalabi A. Birth order and the genetics of amyotrophic lateral sclerosis. J Neurol. 2008 Jan; 255(1):99-102. PMID: 18060566.
      Citations: 1     Fields:    Translation:Humans
    147. Gwinn K, Corriveau RA, Mitsumoto H, Bednarz K, Brown RH, Cudkowicz M, Gordon PH, Hardy J, Kasarskis EJ, Kaufmann P, Miller R, Sorenson E, Tandan R, Traynor BJ, Nash J, Sherman A, Mailman MD, Ostell J, Bruijn L, Cwik V, Rich SS, Singleton A, Refolo L, Andrews J, Zhang R, Conwit R, Keller MA. Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery. PLoS One. 2007 Dec 05; 2(12):e1254. PMID: 18060051.
      Citations: 5     Fields:    Translation:HumansCells
    148. Ingelsson M, Ramasamy K, Russ C, Freeman SH, Orne J, Raju S, Matsui T, Growdon JH, Frosch MP, Ghetti B, Brown RH, Irizarry MC, Hyman BT. Increase in the relative expression of tau with four microtubule binding repeat regions in frontotemporal lobar degeneration and progressive supranuclear palsy brains. Acta Neuropathol. 2007 Nov; 114(5):471-9. PMID: 17721707.
      Citations: 18     Fields:    Translation:HumansCells
    149. Fogh I, Rijsdijk F, Andersen PM, Sham PC, Knight J, Neale B, McKenna-Yasek D, Silani V, Brown RH, Powell JF, Al-Chalabi A. Age at onset in sod1-mediated amyotrophic lateral sclerosis shows familiality. Neurogenetics. 2007 Aug; 8(3):235-6. PMID: 17549529.
      Citations: 10     Fields:    Translation:Humans
    150. Glover L, Brown RH. Dysferlin in membrane trafficking and patch repair. Traffic. 2007 Jul; 8(7):785-94. PMID: 17547707.
      Citations: 72     Fields:    Translation:HumansAnimalsCells
    151. Watanabe S, Nagano S, Duce J, Kiaei M, Li QX, Tucker SM, Tiwari A, Brown RH, Beal MF, Hayward LJ, Culotta VC, Yoshihara S, Sakoda S, Bush AI. Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis. Free Radic Biol Med. 2007 May 15; 42(10):1534-42. PMID: 17448900.
      Citations: 14     Fields:    Translation:HumansAnimalsCells
    152. Brown RH, Grant PE, Pierson CR. Case records of the Massachusetts General Hospital. Case 35-2006. A newborn boy with hypotonia. N Engl J Med. 2006 Nov 16; 355(20):2132-42. PMID: 17108346.
      Citations: 1     Fields:    Translation:Humans
    153. Shefner JM, Cudkowicz M, Brown RH. Motor unit number estimation predicts disease onset and survival in a transgenic mouse model of amyotrophic lateral sclerosis. Muscle Nerve. 2006 Nov; 34(5):603-7. PMID: 16892429.
      Citations: 22     Fields:    Translation:HumansAnimalsCells
    154. Larsen KE, Benn SC, Ay I, Chian RJ, Celia SA, Remington MP, Bejarano M, Liu M, Ross J, Carmillo P, Sah D, Phillips KA, Sulzer D, Pepinsky RB, Fishman PS, Brown RH, Francis JW. A glial cell line-derived neurotrophic factor (GDNF):tetanus toxin fragment C protein conjugate improves delivery of GDNF to spinal cord motor neurons in mice. Brain Res. 2006 Nov 20; 1120(1):1-12. PMID: 17020749.
      Citations: 13     Fields:    Translation:HumansAnimalsCells
    155. Broom WJ, Auwarter KE, Ni J, Russel DE, Yeh LA, Maxwell MM, Glicksman M, Kazantsev AG, Brown RH. Two approaches to drug discovery in SOD1-mediated ALS. J Biomol Screen. 2006 Oct; 11(7):729-35. PMID: 16928982.
      Citations: 10     Fields:    Translation:HumansAnimalsCells
    156. O'Neill GN, Gonzalez RG, Cros DP, Ackerman RH, Brown RH, Stemmer-Rachamimov A. Case records of the Massachusetts General Hospital. Case 22-2006--a 77-year-old man with a rapidly progressive gait disorder. N Engl J Med. 2006 Jul 20; 355(3):296-304. PMID: 16855271.
      Citations:    Fields:    Translation:HumansCells
    157. Triggs WJ, Brown RH, Menkes DL. Case records of the Massachusetts General Hospital. Case 18-2006. A 57-year-old woman with numbness and weakness of the feet and legs. N Engl J Med. 2006 Jun 15; 354(24):2584-92. PMID: 16775239.
      Citations: 5     Fields:    Translation:HumansCells
    158. Boston-Howes W, Gibb SL, Williams EO, Pasinelli P, Brown RH, Trotti D. Caspase-3 cleaves and inactivates the glutamate transporter EAAT2. J Biol Chem. 2006 May 19; 281(20):14076-84. PMID: 16567804.
      Citations: 63     Fields:    Translation:HumansAnimalsCells
    159. Shah PR, Ahmad-Annuar A, Ahmadi KR, Russ C, Sapp PC, Horvitz HR, Brown RH, Goldstein DB, Fisher EM. No association of DYNC1H1 with sporadic ALS in a case-control study of a northern European derived population: a tagging SNP approach. Amyotroph Lateral Scler. 2006 Mar; 7(1):46-56. PMID: 16546759.
      Citations: 3     Fields:    Translation:Humans
    160. Hadano S, Benn SC, Kakuta S, Otomo A, Sudo K, Kunita R, Suzuki-Utsunomiya K, Mizumura H, Shefner JM, Cox GA, Iwakura Y, Brown RH, Ikeda JE. Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking. Hum Mol Genet. 2006 Jan 15; 15(2):233-50. PMID: 16321985.
      Citations: 50     Fields:    Translation:AnimalsCells
    161. Benn SC, Ay I, Bastia E, Chian RJ, Celia SA, Pepinsky RB, Fishman PS, Brown RH, Francis JW. Tetanus toxin fragment C fusion facilitates protein delivery to CNS neurons from cerebrospinal fluid in mice. J Neurochem. 2005 Nov; 95(4):1118-31. PMID: 16271047.
      Citations: 8     Fields:    Translation:HumansAnimalsCells
    162. McCampbell A, Truong D, Broom DC, Allchorne A, Gable K, Cutler RG, Mattson MP, Woolf CJ, Frosch MP, Harmon JM, Dunn TM, Brown RH. Mutant SPTLC1 dominantly inhibits serine palmitoyltransferase activity in vivo and confers an age-dependent neuropathy. Hum Mol Genet. 2005 Nov 15; 14(22):3507-21. PMID: 16210380.
      Citations: 37     Fields:    Translation:AnimalsCells
    163. Broom WJ, Russ C, Sapp PC, McKenna-Yasek D, Hosler BA, Andersen PM, Brown RH. Variants in candidate ALS modifier genes linked to Cu/Zn superoxide dismutase do not explain divergent survival phenotypes. Neurosci Lett. 2006 Jan 09; 392(1-2):52-7. PMID: 16174551.
      Citations: 4     Fields:    Translation:HumansAnimalsCells
    164. Ryu H, Smith K, Camelo SI, Carreras I, Lee J, Iglesias AH, Dangond F, Cormier KA, Cudkowicz ME, Brown RH, Ferrante RJ. Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice. J Neurochem. 2005 Jun; 93(5):1087-98. PMID: 15934930.
      Citations: 142     Fields:    Translation:AnimalsCells
    165. Brown RH. Amyotrophic lateral sclerosis--a new role for old drugs. N Engl J Med. 2005 Mar 31; 352(13):1376-8. PMID: 15800236.
      Citations: 3     Fields:    Translation:Animals
    166. Ray SS, Nowak RJ, Brown RH, Lansbury PT. Small-molecule-mediated stabilization of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants against unfolding and aggregation. Proc Natl Acad Sci U S A. 2005 Mar 08; 102(10):3639-44. PMID: 15738401.
      Citations: 56     Fields:    Translation:HumansCells
    167. Rozen S, Cudkowicz ME, Bogdanov M, Matson WR, Kristal BS, Beecher C, Harrison S, Vouros P, Flarakos J, Vigneau-Callahan K, Matson TD, Newhall KM, Beal MF, Brown RH, Kaddurah-Daouk R. Metabolomic analysis and signatures in motor neuron disease. Metabolomics. 2005; 1(2):101-108. PMID: 18820733.
      Citations: 67     Fields:    
    168. Li YJ, Pericak-Vance MA, Haines JL, Siddique N, McKenna-Yasek D, Hung WY, Sapp P, Allen CI, Chen W, Hosler B, Saunders AM, Dellefave LM, Brown RH, Siddique T. Apolipoprotein E is associated with age at onset of amyotrophic lateral sclerosis. Neurogenetics. 2004 Dec; 5(4):209-13. PMID: 15657798.
      Citations: 27     Fields:    Translation:Humans
    169. Brown RH, Taenkhum K, Buckley TJ, Hamilton RG. Different latex aeroallergen size distributions between powdered surgical and examination gloves: significance for environmental avoidance. J Allergy Clin Immunol. 2004 Aug; 114(2):358-63. PMID: 15316516.
      Citations: 3     Fields:    Translation:HumansPHPublic Health
    170. Ho M, Post CM, Donahue LR, Lidov HG, Bronson RT, Goolsby H, Watkins SC, Cox GA, Brown RH. Disruption of muscle membrane and phenotype divergence in two novel mouse models of dysferlin deficiency. Hum Mol Genet. 2004 Sep 15; 13(18):1999-2010. PMID: 15254015.
      Citations: 105     Fields:    Translation:HumansAnimalsCells
    171. Pasinelli P, Belford ME, Lennon N, Bacskai BJ, Hyman BT, Trotti D, Brown RH. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron. 2004 Jul 08; 43(1):19-30. PMID: 15233914.
      Citations: 190     Fields:    Translation:HumansAnimalsCells
    172. Zhu S, Li M, Figueroa BE, Liu A, Stavrovskaya IG, Pasinelli P, Beal MF, Brown RH, Kristal BS, Ferrante RJ, Friedlander RM. Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in mice. J Neurosci. 2004 Jun 30; 24(26):5909-12. PMID: 15229238.
      Citations: 32     Fields:    Translation:AnimalsCells
    173. Ray SS, Nowak RJ, Strokovich K, Brown RH, Walz T, Lansbury PT. An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis. Biochemistry. 2004 May 04; 43(17):4899-905. PMID: 15109247.
      Citations: 53     Fields:    Translation:HumansCells
    174. Maxwell MM, Pasinelli P, Kazantsev AG, Brown RH. RNA interference-mediated silencing of mutant superoxide dismutase rescues cyclosporin A-induced death in cultured neuroblastoma cells. Proc Natl Acad Sci U S A. 2004 Mar 02; 101(9):3178-83. PMID: 14981234.
      Citations: 25     Fields:    Translation:HumansCells
    175. Kong KY, Ren J, Kraus M, Finklestein SP, Brown RH. Human umbilical cord blood cells differentiate into muscle in sjl muscular dystrophy mice. Stem Cells. 2004; 22(6):981-93. PMID: 15536189.
      Citations: 18     Fields:    Translation:HumansAnimalsCells
    176. Lennon NJ, Kho A, Bacskai BJ, Perlmutter SL, Hyman BT, Brown RH. Dysferlin interacts with annexins A1 and A2 and mediates sarcolemmal wound-healing. J Biol Chem. 2003 Dec 12; 278(50):50466-73. PMID: 14506282.
      Citations: 193     Fields:    Translation:AnimalsCells
    177. Sapp PC, Hosler BA, McKenna-Yasek D, Chin W, Gann A, Genise H, Gorenstein J, Huang M, Sailer W, Scheffler M, Valesky M, Haines JL, Pericak-Vance M, Siddique T, Horvitz HR, Brown RH. Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis. Am J Hum Genet. 2003 Aug; 73(2):397-403. PMID: 12858291.
      Citations: 32     Fields:    Translation:HumansCells
    178. Al-Chalabi A, Scheffler MD, Smith BN, Parton MJ, Cudkowicz ME, Andersen PM, Hayden DL, Hansen VK, Turner MR, Shaw CE, Leigh PN, Brown RH. Ciliary neurotrophic factor genotype does not influence clinical phenotype in amyotrophic lateral sclerosis. Ann Neurol. 2003 Jul; 54(1):130-4. PMID: 12838531.
      Citations: 4     Fields:    Translation:Humans
    179. Andersen PM, Sims KB, Xin WW, Kiely R, O'Neill G, Ravits J, Pioro E, Harati Y, Brower RD, Levine JS, Heinicke HU, Seltzer W, Boss M, Brown RH. Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003 Jun; 4(2):62-73. PMID: 14506936.
      Citations: 81     Fields:    Translation:Humans
    180. Al-Chalabi A, Hansen VK, Simpson CL, Xi J, Hosler BA, Powell JF, McKenna-Yasek D, Shaw CE, Leigh PN, Brown RH. Variants in the ALS2 gene are not associated with sporadic amyotrophic lateral sclerosis. Neurogenetics. 2003 Aug; 4(4):221-2. PMID: 12768434.
      Citations: 2     Fields:    Translation:Humans
    181. Puls I, Jonnakuty C, LaMonte BH, Holzbaur EL, Tokito M, Mann E, Floeter MK, Bidus K, Drayna D, Oh SJ, Brown RH, Ludlow CL, Fischbeck KH. Mutant dynactin in motor neuron disease. Nat Genet. 2003 Apr; 33(4):455-6. PMID: 12627231.
      Citations: 357     Fields:    Translation:HumansAnimalsCells
    182. Hayward LJ, Rodriguez JA, Kim JW, Tiwari A, Goto JJ, Cabelli DE, Valentine JS, Brown RH. Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis. J Biol Chem. 2002 May 03; 277(18):15923-31. PMID: 11854284.
      Citations: 141     Fields:    Translation:HumansAnimalsCells
    183. Shefner JM, Cudkowicz ME, Brown RH. Comparison of incremental with multipoint MUNE methods in transgenic ALS mice. Muscle Nerve. 2002 Jan; 25(1):39-42. PMID: 11754183.
      Citations: 16     Fields:    Translation:AnimalsCells
    184. Ho M, Gallardo E, McKenna-Yasek D, De Luna N, Illa I, Brown RH. A novel, blood-based diagnostic assay for limb girdle muscular dystrophy 2B and Miyoshi myopathy. Ann Neurol. 2002 01; 51(1):129-33. PMID: 11782994.
      Citations: 27     Fields:    Translation:HumansCells
    185. Hand CK, Khoris J, Salachas F, Gros-Louis F, Lopes AA, Mayeux-Portas V, Brewer CG, Brown RH, Meininger V, Camu W, Rouleau GA. A novel locus for familial amyotrophic lateral sclerosis, on chromosome 18q. Am J Hum Genet. 2002 Jan; 70(1):251-6. PMID: 11706389.
      Citations: 34     Fields:    Translation:HumansCells
    Brown's Networks
    Click the
    buttons for more information and interactive visualizations!
    Concepts (710)
    Co-Authors (52)
    Similar People (60)
    Same Department Expand Description
    Physical Neighbors