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    Keywords
    Last Name
    Institution

    Robert H Brown MD, DPhil

    TitleChair and Professor
    InstitutionUniversity of Massachusetts Medical School
    DepartmentNeurology
    AddressUniversity of Massachusetts Medical School
    55 Lake Avenue North
    Worcester MA 01655
    Phone508-334-1271
      Other Positions
      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentBioinformatics and Computational Biology

      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentInterdisciplinary Graduate Program

      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentMasters in Clinical Investigation

      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentMD/PhD Program

      InstitutionUMMS - Graduate School of Biomedical Sciences
      DepartmentNeuroscience

      InstitutionUMMS - Programs, Centers and Institutes
      DepartmentBiochemistry and Molecular Pharmacology

      InstitutionUMMS - Programs, Centers and Institutes
      DepartmentBioinformatics and Integrative Biology

      InstitutionUMMS - Programs, Centers and Institutes
      DepartmentRNA Therapeutics Institute

        Overview 
        Narrative
        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.



        Bibliographic 
        selected publications
        List All   |   Timeline
        1. 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.
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        2. Pickles S, Destroismaisons L, Peyrard SL, Cadot S, Rouleau GA, Brown RH, Julien JP, Arbour N, Vande Velde C. Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet. 2013 Oct 1; 22(19):3947-59.
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        3. 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.
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        4. 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 Feb 12; 4:1472.
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        5. Teng YD, Benn SC, Kalkanis SN, Shefner JM, Onario RC, Cheng B, Lachyankar MB, Marconi M, Li J, Yu D, Han I, Maragakis NJ, Lládo J, Erkmen K, Redmond DE, Sidman RL, Przedborski S, Rothstein JD, Brown RH, Snyder EY. Multimodal Actions of Neural Stem Cells in a Mouse Model of ALS: A Meta-Analysis. Sci Transl Med. 2012 Dec 19; 4(165):165ra164.
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        6. González-Pérez P, Cirulli ET, Drory VE, Dabby R, Nisipeanu P, Carasso RL, Sadeh M, Fox A, Festoff BW, Sapp PC, McKenna-Yasek D, Goldstein DB, Brown RH, Blumen SC. Novel mutation in VCP gene causes atypical amyotrophic lateral sclerosis. Neurology. 2012 Nov 27; 79(22):2201-8.
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        7. 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.
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        8. Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1. Neurobiol Aging. 2013 Jan; 34(1):357.e7-19.
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        9. Van Hoecke A, Schoonaert L, Lemmens R, Timmers M, Staats KA, Laird AS, Peeters E, Philips T, Goris A, Dubois B, Andersen PM, Al-Chalabi A, Thijs V, Turnley AM, van Vught PW, Veldink JH, Hardiman O, Van Den Bosch L, Gonzalez-Perez P, Van Damme P, Brown RH, van den Berg LH, Robberecht W. EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Nat Med. 2012 Sep; 18(9):1418-22.
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        10. 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 22; 488(7412):499-503.
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        11. González-Pérez 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 Jul 2.
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        12. Grose WE, Clark KR, Griffin D, Malik V, Shontz KM, Montgomery CL, Lewis S, Brown RH, Janssen PM, Mendell JR, Rodino-Klapac LR. Homologous recombination mediates functional recovery of dysferlin deficiency following AAV5 gene transfer. PLoS One. 2012; 7(6):e39233.
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        13. 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, Züchner S, Freeman MR. dSarm/Sarm1 is required for activation of an injury-induced axon death pathway. Science. 2012 Jul 27; 337(6093):481-4.
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        14. 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; 4:2801-8.
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        15. 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.
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        16. Ramos EM, Keagle P, Gillis T, Lowe P, Mysore JS, Leclerc AL, Ratti A, Ticozzi N, Gellera C, Gusella JF, Silani V, Alonso I, Brown RH, Macdonald ME, Landers JE. Prevalence of Huntington's disease gene CAG repeat alleles in sporadic amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler. 2012 May; 13(3):265-9.
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        17. 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 1; 121(12):4735-45.
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        18. van Es MA, Schelhaas HJ, van Vught PW, Ticozzi N, Andersen PM, Groen EJ, Schulte C, Blauw HM, Koppers M, Diekstra FP, Fumoto K, Leclerc AL, Keagle P, Bloem BR, Scheffer H, van Nuenen BF, van Blitterswijk M, van Rheenen W, Wills AM, Lowe PP, Hu GF, Yu W, Kishikawa H, Wu D, Folkerth RD, Mariani C, Goldwurm S, Pezzoli G, Van Damme P, Lemmens R, Dahlberg C, Birve A, Fernández-Santiago R, Waibel S, Klein C, Weber M, van der Kooi AJ, de Visser M, Verbaan D, van Hilten JJ, Heutink P, Hennekam EA, Cuppen E, Berg D, Brown RH, Silani V, Gasser T, Ludolph AC, Robberecht W, Ophoff RA, Veldink JH, Pasterkamp RJ, de Bakker PI, Landers JE, van de Warrenburg BP, van den Berg LH. Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol. 2011 Dec; 70(6):964-73.
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        19. 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.
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        20. Krodel DJ, Bittner EA, Abdulnour RE, Brown RH, Eikermann M. Negative pressure pulmonary edema following bronchospasm. Chest. 2011 Nov; 140(5):1351-4.
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        21. Duff RM, Tay V, Hackman P, Ravenscroft G, McLean C, Kennedy P, Steinbach A, Schöffler W, van der Ven PF, Fürst DO, Song J, Djinovic-Carugo K, Penttilä 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):729-40.
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        22. 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.
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        23. Ozdinler PH, Benn S, Yamamoto TH, Güzel M, Brown RH, Macklis JD. Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G93A transgenic ALS mice. J Neurosci. 2011 Mar 16; 31(11):4166-77.
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        24. Ticozzi N, Vance C, Leclerc AL, Keagle P, Glass JD, McKenna-Yasek D, Sapp PC, Silani V, Bosco DA, Shaw CE, Brown RH, Landers JE. Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis. Am J Med Genet B Neuropsychiatr Genet. 2011 Apr; 156B(3):285-90.
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        25. Amato AA, Brown RH. Dysferlinopathies. Handb Clin Neurol. 2011; 101:111-8.
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        26. 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.
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        27. 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, Chiò 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.
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        28. 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.
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        29. 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 1; 19(21):4160-75.
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        30. 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.
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        31. Wright PD, Huang M, Weiss A, Matthews J, Wightman N, Glicksman M, Brown RH. Screening for inhibitors of the SOD1 gene promoter: pyrimethamine does not reduce SOD1 levels in cell and animal models. Neurosci Lett. 2010 Oct 4; 482(3):188-92.
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        32. 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.
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        33. 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.
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        34. Wang B, Yang Z, Brisson BK, Feng H, Zhang Z, Welch EM, Peltz SW, Barton ER, Brown RH, Sweeney HL. Membrane blebbing as an assessment of functional rescue of dysferlin-deficient human myotubes via nonsense suppression. J Appl Physiol. 2010 Sep; 109(3):901-5.
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        35. Taes I, Goris A, Lemmens R, van Es MA, van den Berg LH, Chio A, Traynor BJ, Birve A, Andersen P, Slowik A, Tomik B, Brown RH, Shaw CE, Al-Chalabi A, Boonen S, Van Den Bosch L, Dubois B, Van Damme P, Robberecht W. Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouse. Neurology. 2010 May 25; 74(21):1687-93.
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        36. 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 1; 19(15):2974-86.
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        37. Bogaert E, Goris A, Van Damme P, Geelen V, Lemmens R, van Es MA, van den Berg LH, Sleegers K, Verpoorten N, Timmerman V, De Jonghe P, Van Broeckhoven C, Traynor BJ, Landers JE, Brown RH, Glass JD, Al-Chalabi A, Shaw CE, Birve A, Andersen PM, Slowik A, Tomik B, Melki J, Robberecht W, Van Den Bosch L. Polymorphisms in the GluR2 gene are not associated with amyotrophic lateral sclerosis. Neurobiol Aging. 2012 Feb; 33(2):418-20.
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        38. 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.
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        39. 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.
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        40. 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, Ptácek LJ. Mutations in potassium channel Kir2.6 cause susceptibility to thyrotoxic hypokalemic periodic paralysis. Cell. 2010 Jan 8; 140(1):88-98.
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        41. Ticozzi N, Leclerc AL, van Blitterswijk M, Keagle P, McKenna-Yasek DM, Sapp PC, Silani V, Wills AM, Brown RH, Landers JE. Mutational analysis of TARDBP in neurodegenerative diseases. Neurobiol Aging. 2011 Nov; 32(11):2096-9.
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        42. Brown RH. Medicine. A reinnervating microRNA. Science. 2009 Dec 11; 326(5959):1494-5.
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        43. 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; 4(12):e8175.
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        44. Eichler FS, Hornemann T, McCampbell A, Kuljis D, Penno A, Vardeh D, Tamrazian E, Garofalo K, Lee HJ, Kini L, Selig M, Frosch M, Gable K, von Eckardstein A, Woolf CJ, Guan G, Harmon JM, Dunn TM, Brown RH. Overexpression of the wild-type SPT1 subunit lowers desoxysphingolipid levels and rescues the phenotype of HSAN1. J Neurosci. 2009 Nov 18; 29(46):14646-51.
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        45. 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.
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        46. 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.
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        47. 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 1; 23(19):2294-306.
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        48. Ticozzi N, Silani V, LeClerc AL, Keagle P, Gellera C, Ratti A, Taroni F, Kwiatkowski TJ, McKenna-Yasek DM, Sapp PC, Brown RH, Landers JE. Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort. Neurology. 2009 Oct 13; 73(15):1180-5.
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        49. 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, Nöthen 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.
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        50. Harel NY, Cudkowicz ME, Brown RH, Strittmatter SM. Serum Nogo-A levels are not elevated in amyotrophic lateral sclerosis patients. Biomarkers. 2009 Sep; 14(6):414-7.
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        51. 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.
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        52. Chian RJ, Li J, Ay I, Celia SA, Kashi BB, Tamrazian E, Matthews JC, Bronson RT, Rossomando A, Pepinsky RB, Fishman PS, Brown RH, Francis JW. IGF-1:tetanus toxin fragment C fusion protein improves delivery of IGF-1 to spinal cord but fails to prolong survival of ALS mice. Brain Res. 2009 Sep 1; 1287:1-19.
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        53. 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.
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        54. 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 2; 106(22):9004-9.
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        55. Cudkowicz ME, Andres PL, Macdonald SA, Bedlack RS, Choudry R, Brown RH, Zhang H, Schoenfeld DA, Shefner J, Matson S, Matson WR, Ferrante RJ. Phase 2 study of sodium phenylbutyrate in ALS. Amyotroph Lateral Scler. 2009 Apr; 10(2):99-106.
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        56. Wills AM, Cronin S, Slowik A, Kasperaviciute D, Van Es MA, Morahan JM, Valdmanis PN, Meininger V, Melki J, Shaw CE, Rouleau GA, Fisher EM, Shaw PJ, Morrison KE, Pamphlett R, Van den Berg LH, Figlewicz DA, Andersen PM, Al-Chalabi A, Hardiman O, Purcell S, Landers JE, Brown RH. A large-scale international meta-analysis of paraoxonase gene polymorphisms in sporadic ALS. Neurology. 2009 Jul 7; 73(1):16-24.
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        57. Kwiatkowski TJ, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T, Valdmanis P, Rouleau GA, Hosler BA, Cortelli P, de Jong PJ, Yoshinaga Y, Haines JL, Pericak-Vance MA, Yan J, Ticozzi N, Siddique T, McKenna-Yasek D, Sapp PC, Horvitz HR, Landers JE, Brown RH. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science. 2009 Feb 27; 323(5918):1205-8.
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        58. Chow CY, Landers JE, Bergren SK, Sapp PC, Grant AE, Jones JM, Everett L, Lenk GM, McKenna-Yasek DM, Weisman LS, Figlewicz D, Brown RH, Meisler MH. Deleterious variants of FIG4, a phosphoinositide phosphatase, in patients with ALS. Am J Hum Genet. 2009 Jan; 84(1):85-8.
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        59. Caraganis A, Benn S, Cudkowicz M, Brown RH. Thrombopoietin is ineffective in a mouse model of motor neuron disease. Amyotroph Lateral Scler. 2008 Dec; 9(6):354-8.
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        60. Qureshi M, Shui A, Dibernardo AB, Brown RH, Schoenfeld DA, Cudkowicz ME. Medications and laboratory parameters as prognostic factors in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008 Dec; 9(6):369-74.
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        61. 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.
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        62. 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 1; 18(3):472-81.
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        63. 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.
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        64. 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.
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        65. Qureshi M, Brown RH, Rogers JT, Cudkowicz ME. Serum ferritin and metal levels as risk factors for amyotrophic lateral sclerosis. Open Neurol J. 2008; 2:51-4.
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        66. Brown RH. Developmental biology. Neuron research leaps ahead. Science. 2008 Aug 29; 321(5893):1169-70.
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        67. Ay I, Francis JW, Brown RH. VEGF increases blood-brain barrier permeability to Evans blue dye and tetanus toxin fragment C but not adeno-associated virus in ALS mice. Brain Res. 2008 Oct 9; 1234:198-205.
          View in: PubMed
        68. 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.
          View in: PubMed
        69. 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.
          View in: PubMed
        70. 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.
          View in: PubMed
        71. Hayward LJ, Kim JS, Lee MY, Zhou H, Kim JW, Misra K, Salajegheh M, Wu FF, Matsuda C, Reid V, Cros D, Hoffman EP, Renaud JM, Cannon SC, Brown RH. Targeted mutation of mouse skeletal muscle sodium channel produces myotonia and potassium-sensitive weakness. J Clin Invest. 2008 Apr; 118(4):1437-49.
          View in: PubMed
        72. Wills AM, Landers JE, Zhang H, Richter RJ, Caraganis AJ, Cudkowicz ME, Furlong CE, Brown RH. Paraoxonase 1 (PON1) organophosphate hydrolysis is not reduced in ALS. Neurology. 2008 Mar 18; 70(12):929-34.
          View in: PubMed
        73. Landers JE, Leclerc AL, Shi L, Virkud A, Cho T, Maxwell MM, Henry AF, Polak M, Glass JD, Kwiatkowski TJ, Al-Chalabi A, Shaw CE, Leigh PN, Rodriguez-Leyza I, McKenna-Yasek D, Sapp PC, Brown RH. New VAPB deletion variant and exclusion of VAPB mutations in familial ALS. Neurology. 2008 Apr 1; 70(14):1179-85.
          View in: PubMed
        74. 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.
          View in: PubMed
        75. McCormick AL, Brown RH, Cudkowicz ME, Al-Chalabi A, Garson JA. Quantification of reverse transcriptase in ALS and elimination of a novel retroviral candidate. Neurology. 2008 Jan 22; 70(4):278-83.
          View in: PubMed
        76. 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.
          View in: PubMed
        77. 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; 2(12):e1254.
          View in: PubMed
        78. Broom WJ, Johnson DV, Auwarter KE, Iafrate AJ, Russ C, Al-Chalabi A, Sapp PC, McKenna-Yasek D, Andersen PM, Brown RH. SOD1A4V-mediated ALS: absence of a closely linked modifier gene and origination in Asia. Neurosci Lett. 2008 Jan 17; 430(3):241-5.
          View in: PubMed
        79. Niemann S, Broom WJ, Brown RH. Analysis of a genetic defect in the TATA box of the SOD1 gene in a patient with familial amyotrophic lateral sclerosis. Muscle Nerve. 2007 Nov; 36(5):704-7.
          View in: PubMed
        80. Townsend SA, Evrony GD, Gu FX, Schulz MP, Brown RH, Langer R. Tetanus toxin C fragment-conjugated nanoparticles for targeted drug delivery to neurons. Biomaterials. 2007 Dec; 28(34):5176-84.
          View in: PubMed
        81. Gibb SL, Boston-Howes W, Lavina ZS, Gustincich S, Brown RH, Pasinelli P, Trotti D. A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. J Biol Chem. 2007 Nov 2; 282(44):32480-90.
          View in: PubMed
        82. Niemann S, Kanki H, Fukui Y, Takao K, Fukaya M, Hynynen MN, Churchill MJ, Shefner JM, Bronson RT, Brown RH, Watanabe M, Miyakawa T, Itohara S, Hayashi Y. Genetic ablation of NMDA receptor subunit NR3B in mouse reveals motoneuronal and nonmotoneuronal phenotypes. Eur J Neurosci. 2007 Sep; 26(6):1407-20.
          View in: PubMed
        83. 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.
          View in: PubMed
        84. Niemann S, Landers JE, Churchill MJ, Hosler B, Sapp P, Speed WC, Lahn BT, Kidd KK, Brown RH, Hayashi Y. Motoneuron-specific NR3B gene: no association with ALS and evidence for a common null allele. Neurology. 2008 Feb 26; 70(9):666-76.
          View in: PubMed
        85. Portera-Cailliau C, Russ C, Brown RH, Budson AE, Vonsattel JP, Folkerth RD, Corbo JC. A familial form of pallidoluysionigral degeneration and amyotrophic lateral sclerosis with divergent clinical presentations. J Neuropathol Exp Neurol. 2007 Jul; 66(7):650-9.
          View in: PubMed
        86. 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.
          View in: PubMed
        87. Glover L, Brown RH. Dysferlin in membrane trafficking and patch repair. Traffic. 2007 Jul; 8(7):785-94.
          View in: PubMed
        88. Hetz C, Thielen P, Fisher J, Pasinelli P, Brown RH, Korsmeyer S, Glimcher L. The proapoptotic BCL-2 family member BIM mediates motoneuron loss in a model of amyotrophic lateral sclerosis. Cell Death Differ. 2007 Jul; 14(7):1386-9.
          View in: PubMed
        89. 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.
          View in: PubMed
        90. 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.
          View in: PubMed
        91. 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.
          View in: PubMed
        92. 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.
          View in: PubMed
        93. Pasinelli P, Brown RH. Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat Rev Neurosci. 2006 Sep; 7(9):710-23.
          View in: PubMed
        94. Qureshi MM, Hayden D, Urbinelli L, Ferrante K, Newhall K, Myers D, Hilgenberg S, Smart R, Brown RH, Cudkowicz ME. Analysis of factors that modify susceptibility and rate of progression in amyotrophic lateral sclerosis (ALS). Amyotroph Lateral Scler. 2006 Sep; 7(3):173-82.
          View in: PubMed
        95. 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.
          View in: PubMed
        96. 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.
          View in: PubMed
        97. Brown RH, Amato A. Calpainopathy and eosinophilic myositis. Ann Neurol. 2006 Jun; 59(6):875-7.
          View in: PubMed
        98. 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.
          View in: PubMed
        99. 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.
          View in: PubMed
        100. Greenway MJ, Andersen PM, Russ C, Ennis S, Cashman S, Donaghy C, Patterson V, Swingler R, Kieran D, Prehn J, Morrison KE, Green A, Acharya KR, Brown RH, Hardiman O. ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis. Nat Genet. 2006 Apr; 38(4):411-3.
          View in: PubMed
        101. Pasinetti GM, Ungar LH, Lange DJ, Yemul S, Deng H, Yuan X, Brown RH, Cudkowicz ME, Newhall K, Peskind E, Marcus S, Ho L. Identification of potential CSF biomarkers in ALS. Neurology. 2006 Apr 25; 66(8):1218-22.
          View in: PubMed
        102. Morita M, Al-Chalabi A, Andersen PM, Hosler B, Sapp P, Englund E, Mitchell JE, Habgood JJ, de Belleroche J, Xi J, Jongjaroenprasert W, Horvitz HR, Gunnarsson LG, Brown RH. A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia. Neurology. 2006 Mar 28; 66(6):839-44.
          View in: PubMed
        103. 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.
          View in: PubMed
        104. Ranganathan S, Williams E, Ganchev P, Gopalakrishnan V, Lacomis D, Urbinelli L, Newhall K, Cudkowicz ME, Brown RH, Bowser R. Proteomic profiling of cerebrospinal fluid identifies biomarkers for amyotrophic lateral sclerosis. J Neurochem. 2005 Dec; 95(5):1461-71.
          View in: PubMed
        105. 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.
          View in: PubMed
        106. Broom WJ, Ay I, Pasinelli P, Brown RH. Inhibition of SOD1 expression by mitomycin C is a non-specific consequence of cellular toxicity. Neurosci Lett. 2006 Jan 30; 393(2-3):184-8.
          View in: PubMed
        107. 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.
          View in: PubMed
        108. 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 9; 392(1-2):52-7.
          View in: PubMed
        109. Garcia ML, Singleton AB, Hernandez D, Ward CM, Evey C, Sapp PA, Hardy J, Brown RH, Cleveland DW. Mutations in neurofilament genes are not a significant primary cause of non-SOD1-mediated amyotrophic lateral sclerosis. Neurobiol Dis. 2006 Jan; 21(1):102-9.
          View in: PubMed
        110. 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.
          View in: PubMed
        111. Brown RH. Amyotrophic lateral sclerosis--a new role for old drugs. N Engl J Med. 2005 Mar 31; 352(13):1376-8.
          View in: PubMed
        112. 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 8; 102(10):3639-44.
          View in: PubMed
        113. Steele AJ, Al-Chalabi A, Ferrante K, Cudkowicz ME, Brown RH, Garson JA. Detection of serum reverse transcriptase activity in patients with ALS and unaffected blood relatives. Neurology. 2005 Feb 8; 64(3):454-8.
          View in: PubMed
        114. 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.
          View in: PubMed
        115. 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.
          View in: PubMed
        116. 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 8; 43(1):19-30.
          View in: PubMed
        117. 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.
          View in: PubMed
        118. Francis JW, Bastia E, Matthews CC, Parks DA, Schwarzschild MA, Brown RH, Fishman PS. Tetanus toxin fragment C as a vector to enhance delivery of proteins to the CNS. Brain Res. 2004 Jun 11; 1011(1):7-13.
          View in: PubMed
        119. 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 4; 43(17):4899-905.
          View in: PubMed
        120. Nix WA, Berger MM, Oberste MS, Brooks BR, McKenna-Yasek DM, Brown RH, Roos RP, Pallansch MA. Failure to detect enterovirus in the spinal cord of ALS patients using a sensitive RT-PCR method. Neurology. 2004 Apr 27; 62(8):1372-7.
          View in: PubMed
        121. Benn SC, Brown RH. Putting the heat on ALS. Nat Med. 2004 Apr; 10(4):345-7.
          View in: PubMed
        122. 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 2; 101(9):3178-83.
          View in: PubMed
        123. Dangond F, Hwang D, Camelo S, Pasinelli P, Frosch MP, Stephanopoulos G, Stephanopoulos G, Brown RH, Gullans SR. Molecular signature of late-stage human ALS revealed by expression profiling of postmortem spinal cord gray matter. Physiol Genomics. 2004 Jan 15; 16(2):229-39.
          View in: PubMed
        124. Francis JW, Figueiredo D, vanderSpek JC, Ayala LM, Kim YS, Remington MP, Young PJ, Lorson CL, Ikebe S, Fishman PS, Brown RH. A survival motor neuron:tetanus toxin fragment C fusion protein for the targeted delivery of SMN protein to neurons. Brain Res. 2004 Jan 2; 995(1):84-96.
          View in: PubMed
        125. 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.
          View in: PubMed
        126. Clement AM, Nguyen MD, Roberts EA, Garcia ML, Boillée S, Rule M, McMahon AP, Doucette W, Siwek D, Ferrante RJ, Brown RH, Julien JP, Goldstein LS, Cleveland DW. Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science. 2003 Oct 3; 302(5642):113-7.
          View in: PubMed
        127. 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.
          View in: PubMed
        128. Cudkowicz ME, Shefner JM, Schoenfeld DA, Brown RH, Johnson H, Qureshi M, Jacobs M, Rothstein JD, Appel SH, Pascuzzi RM, Heiman-Patterson TD, Donofrio PD, David WS, Russell JA, Tandan R, Pioro EP, Felice KJ, Rosenfeld J, Mandler RN, Sachs GM, Bradley WG, Raynor EM, Baquis GD, Belsh JM, Novella S, Goldstein J, Hulihan J. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurology. 2003 Aug 26; 61(4):456-64.
          View in: PubMed
        129. 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.
          View in: PubMed
        130. 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.
          View in: PubMed
        131. 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.
          View in: PubMed
        132. 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.
          View in: PubMed
        133. 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.
          View in: PubMed
        134. Bejaoui K, Uchida Y, Yasuda S, Ho M, Nishijima M, Brown RH, Holleran WM, Hanada K. Hereditary sensory neuropathy type 1 mutations confer dominant negative effects on serine palmitoyltransferase, critical for sphingolipid synthesis. J Clin Invest. 2002 Nov; 110(9):1301-8.
          View in: PubMed
        135. Cudkowicz ME, Pastusza KA, Sapp PC, Mathews RK, Leahy J, Pasinelli P, Francis JW, Jiang D, Andersen JK, Brown RH. Survival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity. Neurology. 2002 Sep 10; 59(5):729-34.
          View in: PubMed
        136. Morita M, Ho M, Hosler BA, McKenna-Yasek D, Brown RH. A novel mutation in the spastin gene in a family with spastic paraplegia. Neurosci Lett. 2002 May 31; 325(1):57-61.
          View in: PubMed
        137. Soderblom LA, Becker TL, Bennett G, Boice DC, Britt DT, Brown RH, Buratti BJ, Isbell C, Giese B, Hare T, Hicks MD, Howington-Kraus E, Kirk RL, Lee M, Nelson RM, Oberst J, Owen TC, Rayman MD, Sandel BR, Stern SA, Thomas N, Yelle RV. Observations of comet 19P/Borrelly by the miniature integrated camera and spectrometer aboard Deep Space 1. Science. 2002 May 10; 296(5570):1087-91.
          View in: PubMed
        138. 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 3; 277(18):15923-31.
          View in: PubMed
        139. Rodriguez JA, Valentine JS, Eggers DK, Roe JA, Tiwari A, Brown RH, Hayward LJ. Familial amyotrophic lateral sclerosis-associated mutations decrease the thermal stability of distinctly metallated species of human copper/zinc superoxide dismutase. J Biol Chem. 2002 May 3; 277(18):15932-7.
          View in: PubMed
        140. 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.
          View in: PubMed
        141. Dreibelbis JE, Brown RH, Pastuszak KA, Smith ER, Kaplan PL, Cudkowicz ME. Disease course unaltered by a single intracisternal injection of BMP-7 in ALS mice. Muscle Nerve. 2002 Jan; 25(1):122-3.
          View in: PubMed
        142. O'Neill GN, Aoki M, Brown RH. ABCD1 translation-initiator mutation demonstrates genotype-phenotype correlation for AMN. Neurology. 2001 Dec 11; 57(11):1956-62.
          View in: PubMed
        143. Gallardo E, Rojas-García R, de Luna N, Pou A, Brown RH, Illa I. Inflammation in dysferlin myopathy: immunohistochemical characterization of 13 patients. Neurology. 2001 Dec 11; 57(11):2136-8.
          View in: PubMed
        144. Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown RH, Itoyama Y. Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. J Neurosci. 2001 Dec 1; 21(23):9246-54.
          View in: PubMed
        145. Shefner JM, Brown RH, Cole D, Chaturvedi P, Schoenfeld D, Pastuszak K, Matthews R, Upton-Rice M, Cudkowicz ME. Effect of neurophilin ligands on motor units in mice with SOD1 ALS mutations. Neurology. 2001 Nov 27; 57(10):1857-61.
          View in: PubMed
        146. 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.
          View in: PubMed
        147. Hadano S, Hand CK, Osuga H, Yanagisawa Y, Otomo A, Devon RS, Miyamoto N, Showguchi-Miyata J, Okada Y, Singaraja R, Figlewicz DA, Kwiatkowski T, Hosler BA, Sagie T, Skaug J, Nasir J, Brown RH, Scherer SW, Rouleau GA, Hayden MR, Ikeda JE. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet. 2001 Oct; 29(2):166-73.
          View in: PubMed
        148. Matsuda C, Hayashi YK, Ogawa M, Aoki M, Murayama K, Nishino I, Nonaka I, Arahata K, Brown RH. The sarcolemmal proteins dysferlin and caveolin-3 interact in skeletal muscle. Hum Mol Genet. 2001 Aug 15; 10(17):1761-6.
          View in: PubMed
        149. Aoki M, Liu J, Richard I, Bashir R, Britton S, Keers SM, Oeltjen J, Brown HE, Marchand S, Bourg N, Beley C, McKenna-Yasek D, Arahata K, Bohlega S, Cupler E, Illa I, Majneh I, Barohn RJ, Urtizberea JA, Fardeau M, Amato A, Angelini C, Bushby K, Beckmann JS, Brown RH. Genomic organization of the dysferlin gene and novel mutations in Miyoshi myopathy. Neurology. 2001 Jul 24; 57(2):271-8.
          View in: PubMed
        150. Brown RH, Robberecht W. Amyotrophic lateral sclerosis: pathogenesis. Semin Neurol. 2001 Jun; 21(2):131-9.
          View in: PubMed
        151. Bejaoui K, Wu C, Scheffler MD, Haan G, Ashby P, Wu L, de Jong P, Brown RH. SPTLC1 is mutated in hereditary sensory neuropathy, type 1. Nat Genet. 2001 Mar; 27(3):261-2.
          View in: PubMed
        152. Trotti D, Aoki M, Pasinelli P, Berger UV, Danbolt NC, Brown RH, Hediger MA. Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity. J Biol Chem. 2001 Jan 5; 276(1):576-82.
          View in: PubMed
        153. Illa I, Serrano-Munuera C, Gallardo E, Lasa A, Rojas-García R, Palmer J, Gallano P, Baiget M, Matsuda C, Brown RH. Distal anterior compartment myopathy: a dysferlin mutation causing a new muscular dystrophy phenotype. Ann Neurol. 2001 Jan; 49(1):130-4.
          View in: PubMed
        154. Pasinelli P, Houseweart MK, Brown RH, Cleveland DW. Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2000 Dec 5; 97(25):13901-6.
          View in: PubMed
        155. Hosler BA, Siddique T, Sapp PC, Sailor W, Huang MC, Hossain A, Daube JR, Nance M, Fan C, Kaplan J, Hung WY, McKenna-Yasek D, Haines JL, Pericak-Vance MA, Horvitz HR, Brown RH. Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA. 2000 Oct 4; 284(13):1664-9.
          View in: PubMed
        156. Bogdanov M, Brown RH, Matson W, Smart R, Hayden D, O'Donnell H, Flint Beal M, Cudkowicz M. Increased oxidative damage to DNA in ALS patients. Free Radic Biol Med. 2000 Oct 1; 29(7):652-8.
          View in: PubMed
        157. Francis JW, Brown RH, Figueiredo D, Remington MP, Castillo O, Schwarzschild MA, Fishman PS, Murphy JR, vanderSpek JC. Enhancement of diphtheria toxin potency by replacement of the receptor binding domain with tetanus toxin C-fragment: a potential vector for delivering heterologous proteins to neurons. J Neurochem. 2000 Jun; 74(6):2528-36.
          View in: PubMed
        158. Upton-Rice MN, Cudkowicz ME, Warren L, Mathew RK, Ren JM, Finklestein SP, Brown RH. Basic fibroblast growth factor does not prolong survival in a transgenic model of familial amyotrophic lateral sclerosis. Ann Neurol. 1999 Dec; 46(6):934.
          View in: PubMed
        159. Shefner JM, Reaume AG, Flood DG, Scott RW, Kowall NW, Ferrante RJ, Siwek DF, Upton-Rice M, Brown RH. Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy. Neurology. 1999 Oct 12; 53(6):1239-46.
          View in: PubMed
        160. Matsuda C, Aoki M, Hayashi YK, Ho MF, Arahata K, Brown RH. Dysferlin is a surface membrane-associated protein that is absent in Miyoshi myopathy. Neurology. 1999 Sep 22; 53(5):1119-22.
          View in: PubMed
        161. Parboosingh JS, Meininger V, McKenna-Yasek D, Brown RH, Rouleau GA. Deletions causing spinal muscular atrophy do not predispose to amyotrophic lateral sclerosis. Arch Neurol. 1999 Jun; 56(6):710-2.
          View in: PubMed
        162. Lin KI, Pasinelli P, Brown RH, Hardwick JM, Ratan RR. Decreased intracellular superoxide levels activate Sindbis virus-induced apoptosis. J Biol Chem. 1999 May 7; 274(19):13650-5.
          View in: PubMed
        163. Trotti D, Rolfs A, Danbolt NC, Brown RH, Hediger MA. SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter. Nat Neurosci. 1999 May; 2(5):427-33.
          View in: PubMed
        164. Cudkowicz ME, Sexton PM, Ellis T, Hayden DL, Gwilt PR, Whalen J, Brown RH. The pharmacokinetics and pharmaco-dynamics of Procysteine in amyotrophic lateral sclerosis. Neurology. 1999 Apr 22; 52(7):1492-4.
          View in: PubMed
        165. Upton-Rice MN, Cudkowicz ME, Mathew RK, Reif D, Brown RH. Administration of nitric oxide synthase inhibitors does not alter disease course of amyotrophic lateral sclerosis SOD1 mutant transgenic mice. Ann Neurol. 1999 Mar; 45(3):413-4.
          View in: PubMed
        166. Bejaoui K, McKenna-Yasek D, Hosler BA, Burns-Deater E, Deater LM, O'Neill G, Haines JL, Brown RH. Confirmation of linkage of type 1 hereditary sensory neuropathy to human chromosome 9q22. Neurology. 1999 Feb; 52(3):510-5.
          View in: PubMed
        167. Pasinelli P, Borchelt DR, Houseweart MK, Cleveland DW, Brown RH. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase. Proc Natl Acad Sci U S A. 1998 Dec 22; 95(26):15763-8.
          View in: PubMed
        168. Morrill JA, Brown RH, Cannon SC. Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H. J Neurosci. 1998 Dec 15; 18(24):10320-34.
          View in: PubMed
        169. Hosler BA, Sapp PC, Berger R, O'Neill G, Bejaoui K, Hamida MB, Hentati F, Chin W, McKenna-Yasek D, Haines JL, Patterson D, Horvitz HR, Brown RH, Day CB. Refined mapping and characterization of the recessive familial amyotrophic lateral sclerosis locus (ALS2) on chromosome 2q33. Neurogenetics. 1998 Dec; 2(1):34-42.
          View in: PubMed
        170. Brown RH. SOD1 aggregates in ALS: cause, correlate or consequence? Nat Med. 1998 Dec; 4(12):1362-4.
          View in: PubMed
        171. Andreu AL, Bruno C, Shanske S, Shtilbans A, Hirano M, Krishna S, Hayward L, Systrom DS, Brown RH, DiMauro S. Missense mutation in the mtDNA cytochrome b gene in a patient with myopathy. Neurology. 1998 Nov; 51(5):1444-7.
          View in: PubMed
        172. Liu J, Aoki M, Illa I, Wu C, Fardeau M, Angelini C, Serrano C, Urtizberea JA, Hentati F, Hamida MB, Bohlega S, Culper EJ, Amato AA, Bossie K, Oeltjen J, Bejaoui K, McKenna-Yasek D, Hosler BA, Schurr E, Arahata K, de Jong PJ, Brown RH. Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy. Nat Genet. 1998 Sep; 20(1):31-6.
          View in: PubMed
        173. Neumeyer AM, Cros D, McKenna-Yasek D, Zawadzka A, Hoffman EP, Pegoraro E, Hunter RG, Munsat TL, Brown RH. Pilot study of myoblast transfer in the treatment of Becker muscular dystrophy. Neurology. 1998 Aug; 51(2):589-92.
          View in: PubMed
        174. Browne SE, Bowling AC, Baik MJ, Gurney M, Brown RH, Beal MF. Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis. J Neurochem. 1998 Jul; 71(1):281-7.
          View in: PubMed
        175. Cudkowicz ME, McKenna-Yasek D, Chen C, Hedley-Whyte ET, Brown RH. Limited corticospinal tract involvement in amyotrophic lateral sclerosis subjects with the A4V mutation in the copper/zinc superoxide dismutase gene. Ann Neurol. 1998 Jun; 43(6):703-10.
          View in: PubMed
        176. Francis JW, Sandrock AW, Bhide PG, Vonsattel JP, Brown RH. Heterogeneity of subcellular localization and electrophoretic mobility of survival motor neuron (SMN) protein in mammalian neural cells and tissues. Proc Natl Acad Sci U S A. 1998 May 26; 95(11):6492-7.
          View in: PubMed
        177. Aoki M, Lin CL, Rothstein JD, Geller BA, Hosler BA, Munsat TL, Horvitz HR, Brown RH. Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis. Ann Neurol. 1998 May; 43(5):645-53.
          View in: PubMed
        178. Liu J, Wu C, Bossie K, Bejaoui K, Hosler BA, Gingrich JC, Ben Hamida M, Hentati F, Schurr E, de Jong PJ, Brown RH. Generation of a 3-Mb PAC contig spanning the Miyoshi myopathy/limb-girdle muscular dystrophy (MM/LGMD2B) locus on chromosome 2p13. Genomics. 1998 Apr 1; 49(1):23-9.
          View in: PubMed
        179. Bejaoui K, Liu J, McKenna-Yasek D, Le Paslier D, Bossie K, Gilligan DM, Brown RH. Genetic fine mapping of the Miyoshi myopathy locus and exclusion of eight candidate genes. Neurogenetics. 1998 Mar; 1(3):189-96.
          View in: PubMed
        180. Esteban J, Neumeyer AM, McKenna-Yasek D, Brown RH. Identification of two mutations and a polymorphism in the chloride channel CLCN-1 in patients with Becker's generalized myotonia. Neurogenetics. 1998 Mar; 1(3):185-8.
          View in: PubMed
        181. Siddique T, Pericak-Vance MA, Caliendo J, Hong ST, Hung WY, Kaplan J, McKenna-Yasek D, Rimmler JB, Sapp P, Saunders AM, Scott WK, Siddique N, Haines JL, Brown RH. Lack of association between apolipoprotein E genotype and sporadic amyotrophic lateral sclerosis. Neurogenetics. 1998 Mar; 1(3):213-6.
          View in: PubMed
        182. Ferrante RJ, Browne SE, Shinobu LA, Bowling AC, Baik MJ, MacGarvey U, Kowall NW, Brown RH, Beal MF. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis. J Neurochem. 1997 Nov; 69(5):2064-74.
          View in: PubMed
        183. Brown RH. Amyotrophic lateral sclerosis. Insights from genetics. Arch Neurol. 1997 Oct; 54(10):1246-50.
          View in: PubMed
        184. Beal MF, Ferrante RJ, Browne SE, Matthews RT, Kowall NW, Brown RH. Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis. Ann Neurol. 1997 Oct; 42(4):644-54.
          View in: PubMed
        185. Francis JW, Ren J, Warren L, Brown RH, Finklestein SP. Postischemic infusion of Cu/Zn superoxide dismutase or SOD:Tet451 reduces cerebral infarction following focal ischemia/reperfusion in rats. Exp Neurol. 1997 Aug; 146(2):435-43.
          View in: PubMed
        186. Cudkowicz ME, Warren L, Francis JW, Lloyd KJ, Friedlander RM, Borges LF, Kassem N, Munsat TL, Brown RH. Intrathecal administration of recombinant human superoxide dismutase 1 in amyotrophic lateral sclerosis: a preliminary safety and pharmacokinetic study. Neurology. 1997 Jul; 49(1):213-22.
          View in: PubMed
        187. Hayward LJ, Brown RH, Cannon SC. Slow inactivation differs among mutant Na channels associated with myotonia and periodic paralysis. Biophys J. 1997 Mar; 72(3):1204-19.
          View in: PubMed
        188. Cudkowicz ME, McKenna-Yasek D, Sapp PE, Chin W, Geller B, Hayden DL, Schoenfeld DA, Hosler BA, Horvitz HR, Brown RH. Epidemiology of mutations in superoxide dismutase in amyotrophic lateral sclerosis. Ann Neurol. 1997 Feb; 41(2):210-21.
          View in: PubMed
        189. Brown RH. Dystrophin-associated proteins and the muscular dystrophies. Annu Rev Med. 1997; 48:457-66.
          View in: PubMed
        190. Hosler BA, Brown RH. Superoxide dismutase and oxygen radical neurotoxicity. Curr Opin Neurol. 1996 Dec; 9(6):486-91.
          View in: PubMed
        191. Przedborski S, Dhawan V, Donaldson DM, Murphy PL, McKenna-Yasek D, Mandel FS, Brown RH, Eidelberg D. Nigrostriatal dopaminergic function in familial amyotrophic lateral sclerosis patients with and without copper/zinc superoxide dismutase mutations. Neurology. 1996 Dec; 47(6):1546-51.
          View in: PubMed
        192. Brown RH, Miller JB. Progress, problems, and prospects for gene therapy in muscle. Curr Opin Rheumatol. 1996 Nov; 8(6):539-43.
          View in: PubMed
        193. Hosler BA, Nicholson GA, Sapp PC, Chin W, Orrell RW, de Belleroche JS, Esteban J, Hayward LJ, Mckenna-Yasek D, Yeung L, Cherryson AK, Dench JE, Wilton SD, Laing NG, Horvitz HR, Brown RH. Three novel mutations and two variants in the gene for Cu/Zn superoxide dismutase in familial amyotrophic lateral sclerosis. Neuromuscul Disord. 1996 Oct; 6(5):361-6.
          View in: PubMed
        194. Vechio JD, Bruijn LI, Xu Z, Brown RH, Cleveland DW. Sequence variants in human neurofilament proteins: absence of linkage to familial amyotrophic lateral sclerosis. Ann Neurol. 1996 Oct; 40(4):603-10.
          View in: PubMed
        195. Cudkowicz ME, Brown RH. An update on superoxide dismutase 1 in familial amyotrophic lateral sclerosis. J Neurol Sci. 1996 Aug; 139 Suppl:10-5.
          View in: PubMed
        196. Reaume AG, Elliott JL, Hoffman EK, Kowall NW, Ferrante RJ, Siwek DF, Wilcox HM, Flood DG, Beal MF, Brown RH, Scott RW, Snider WD. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat Genet. 1996 May; 13(1):43-7.
          View in: PubMed
        197. Hayward LJ, Brown RH, Cannon SC. Inactivation defects caused by myotonia-associated mutations in the sodium channel III-IV linker. J Gen Physiol. 1996 May; 107(5):559-76.
          View in: PubMed
        198. Przedborski S, Donaldson DM, Murphy PL, Hirsch O, Lange D, Naini AB, McKenna-Yasek D, Brown RH. Blood superoxide dismutase, catalase and glutathione peroxidase activities in familial and sporadic amyotrophic lateral sclerosis. Neurodegeneration. 1996 Mar; 5(1):57-64.
          View in: PubMed
        199. Brown RH. Superoxide dismutase and familial amyotrophic lateral sclerosis: new insights into mechanisms and treatments. Ann Neurol. 1996 Feb; 39(2):145-6.
          View in: PubMed
        200. Brown RH. Dystrophin-associated proteins and the muscular dystrophies: a glossary. Brain Pathol. 1996 Jan; 6(1):19-24.
          View in: PubMed
        201. Grosson CL, Esteban J, McKenna-Yasek D, Gusella JF, Brown RH. Hypokalemic periodic paralysis mutations: confirmation of mutation and analysis of founder effect. Neuromuscul Disord. 1996 Jan; 6(1):27-31.
          View in: PubMed
        202. Williamson TL, Marszalek JR, Vechio JD, Bruijn LI, Lee MK, Xu Z, Brown RH, Cleveland DW. Neurofilaments, radial growth of axons, and mechanisms of motor neuron disease. Cold Spring Harb Symp Quant Biol. 1996; 61:709-23.
          View in: PubMed
        203. Brown RH. Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function. Curr Opin Neurobiol. 1995 Dec; 5(6):841-6.
          View in: PubMed
        204. Sapp PC, Rosen DR, Hosler BA, Esteban J, McKenna-Yasek D, O'Regan JP, Horvitz HR, Brown RH. Identification of three novel mutations in the gene for Cu/Zn superoxide dismutase in patients with familial amyotrophic lateral sclerosis. Neuromuscul Disord. 1995 Sep; 5(5):353-7.
          View in: PubMed
        205. Mui S, Rebeck GW, McKenna-Yasek D, Hyman BT, Brown RH. Apolipoprotein E epsilon 4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis. Ann Neurol. 1995 Sep; 38(3):460-3.
          View in: PubMed
        206. Francis JW, Hosler BA, Brown RH, Fishman PS. CuZn superoxide dismutase (SOD-1):tetanus toxin fragment C hybrid protein for targeted delivery of SOD-1 to neuronal cells. J Biol Chem. 1995 Jun 23; 270(25):15434-42.
          View in: PubMed
        207. Bowling AC, Barkowski EE, McKenna-Yasek D, Sapp P, Horvitz HR, Beal MF, Brown RH. Superoxide dismutase concentration and activity in familial amyotrophic lateral sclerosis. J Neurochem. 1995 May; 64(5):2366-9.
          View in: PubMed
        208. Cannon SC, Hayward LJ, Beech J, Brown RH. Sodium channel inactivation is impaired in equine hyperkalemic periodic paralysis. J Neurophysiol. 1995 May; 73(5):1892-9.
          View in: PubMed
        209. Brown RH. Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice. Cell. 1995 Mar 10; 80(5):687-92.
          View in: PubMed
        210. Pramatarova A, Figlewicz DA, Krizus A, Han FY, Ceballos-Picot I, Nicole A, Dib M, Meininger V, Brown RH, Rouleau GA. Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis. Am J Hum Genet. 1995 Mar; 56(3):592-6.
          View in: PubMed
        211. Parboosingh JS, Rouleau GA, Meninger V, McKenna-Yasek D, Brown RH, Figlewicz DA. Absence of mutations in the Mn superoxide dismutase or catalase genes in familial amyotrophic lateral sclerosis. Neuromuscul Disord. 1995 Jan; 5(1):7-10.
          View in: PubMed
        212. Rosen DR, Trofatter JA, Brown RH. Mapping of the human TATA-binding protein gene (TBP) to chromosome 6qter. Cytogenet Cell Genet. 1995; 69(3-4):279-80.
          View in: PubMed
        213. Hosler BA, Brown RH. Copper/zinc superoxide dismutase mutations and free radical damage in amyotrophic lateral sclerosis. Adv Neurol. 1995; 68:41-6.
          View in: PubMed
        214. Brown RH. A transgenic-mouse model of amyotrophic lateral sclerosis. N Engl J Med. 1994 Oct 20; 331(16):1091-2.
          View in: PubMed
        215. Borchelt DR, Lee MK, Slunt HS, Guarnieri M, Xu ZS, Wong PC, Brown RH, Price DL, Sisodia SS, Cleveland DW. Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity. Proc Natl Acad Sci U S A. 1994 Aug 16; 91(17):8292-6.
          View in: PubMed
        216. Figlewicz DA, McInnis MG, Goto J, Haines JL, Warren AC, Krizus A, Khodr N, Brown RH, McKenna-Yasek D, Antonarakis SE, et al. Identification of flanking markers for the familial amyotrophic lateral sclerosis gene ALS1 on chromosome 21. J Neurol Sci. 1994 Jul; 124 Suppl:90-5.
          View in: PubMed
        217. Wirguin I, Suturkova-Milosevic L, Della-Latta P, Fisher T, Brown RH, Latov N. Monoclonal IgM antibodies to GM1 and asialo-GM1 in chronic neuropathies cross-react with Campylobacter jejuni lipopolysaccharides. Ann Neurol. 1994 Jun; 35(6):698-703.
          View in: PubMed
        218. Esteban J, Rosen DR, Bowling AC, Sapp P, McKenna-Yasek D, O'Regan JP, Beal MF, Horvitz HR, Brown RH. Identification of two novel mutations and a new polymorphism in the gene for Cu/Zn superoxide dismutase in patients with amyotrophic lateral sclerosis. Hum Mol Genet. 1994 Jun; 3(6):997-8.
          View in: PubMed
        219. Rosen DR, Sapp P, O'Regan J, McKenna-Yasek D, Schlumpf KS, Haines JL, Gusella JF, Horvitz HR, Brown RH. Genetic linkage analysis of familial amyotrophic lateral sclerosis using human chromosome 21 microsatellite DNA markers. Am J Med Genet. 1994 May 15; 51(1):61-9.
          View in: PubMed
        220. Rothstein JD, Bristol LA, Hosler B, Brown RH, Kuncl RW. Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons. Proc Natl Acad Sci U S A. 1994 May 10; 91(10):4155-9.
          View in: PubMed
        221. D'Amore PA, Brown RH, Ku PT, Hoffman EP, Watanabe H, Arahata K, Ishihara T, Folkman J. Elevated basic fibroblast growth factor in the serum of patients with Duchenne muscular dystrophy. Ann Neurol. 1994 Mar; 35(3):362-5.
          View in: PubMed
        222. Brown RH. Ion channel mutations in periodic paralysis and related myotonic diseases. Ann N Y Acad Sci. 1993 Dec 20; 707:305-16.
          View in: PubMed
        223. Bowling AC, Schulz JB, Brown RH, Beal MF. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis. J Neurochem. 1993 Dec; 61(6):2322-5.
          View in: PubMed
        224. Hardiman O, Sklar RM, Brown RH. Direct effects of cyclosporin A and cyclophosphamide on differentiation of normal human myoblasts in culture. Neurology. 1993 Jul; 43(7):1432-4.
          View in: PubMed
        225. Cannon SC, Brown RH, Corey DP. Theoretical reconstruction of myotonia and paralysis caused by incomplete inactivation of sodium channels. Biophys J. 1993 Jul; 65(1):270-88.
          View in: PubMed
        226. Rosen DR, Brown RH. Dinucleotide repeat polymorphism in the HOX4E locus. Hum Mol Genet. 1993 May; 2(5):617.
          View in: PubMed
        227. Hardiman O, Halperin JJ, Farrell MA, Shapiro BE, Wray SH, Brown RH. Neuropathic findings in oculopharyngeal muscular dystrophy. A report of seven cases and a review of the literature. Arch Neurol. 1993 May; 50(5):481-8.
          View in: PubMed
        228. Lev AA, Rosen DR, Kos C, Clifford E, Landes G, Hauser SL, Brown RH. Human ciliary neurotrophic factor: localization to the proximal region of the long arm of chromosome 11 and association with CA/GT dinucleotide repeat. Genomics. 1993 May; 16(2):539-41.
          View in: PubMed
        229. Goto J, Figlewicz DA, Haines JL, Brown RH, Khodr N, Rouleau GA. The glycinamide ribonucleotide transformylase (GART) gene is not responsible for familial amyotrophic lateral sclerosis. Neuromuscul Disord. 1993 Mar; 3(2):157-60.
          View in: PubMed
        230. Hardiman O, Faustman D, Li X, Sklar RM, Brown RH. Expression of major histocompatibility complex antigens in cultures of clonally derived human myoblasts. Neurology. 1993 Mar; 43(3 Pt 1):604-8.
          View in: PubMed
        231. Hardiman O, Sklar RM, Brown RH. Methylprednisolone selectively affects dystrophin expression in human muscle cultures. Neurology. 1993 Feb; 43(2):342-5.
          View in: PubMed
        232. Neumeyer AM, DiGregorio DM, Brown RH. Arterial delivery of myoblasts to skeletal muscle. Neurology. 1992 Dec; 42(12):2258-62.
          View in: PubMed
        233. McClatchey AI, McKenna-Yasek D, Cros D, Worthen HG, Kuncl RW, DeSilva SM, Cornblath DR, Gusella JF, Brown RH. Novel mutations in families with unusual and variable disorders of the skeletal muscle sodium channel. Nat Genet. 1992 Oct; 2(2):148-52.
          View in: PubMed
        234. Hardiman O, Brown RH, Beggs AH, Specht L, Sklar RM. Differential glucocorticoid effects on the fusion of Duchenne/Becker and control muscle cultures: pharmacologic detection of accelerated aging in dystrophic muscle. Neurology. 1992 May; 42(5):1085-91.
          View in: PubMed
        235. McClatchey AI, Van den Bergh P, Pericak-Vance MA, Raskind W, Verellen C, McKenna-Yasek D, Rao K, Haines JL, Bird T, Brown RH, et al. Temperature-sensitive mutations in the III-IV cytoplasmic loop region of the skeletal muscle sodium channel gene in paramyotonia congenita. Cell. 1992 Feb 21; 68(4):769-74.
          View in: PubMed
        236. Hoffman EP, Brown RH, Kunkel LM. Dystrophin: the protein product of the Duchene muscular dystrophy locus. 1987. Biotechnology. 1992; 24:457-66.
          View in: PubMed
        237. Rojas CV, Wang JZ, Schwartz LS, Hoffman EP, Powell BR, Brown RH. A Met-to-Val mutation in the skeletal muscle Na+ channel alpha-subunit in hyperkalaemic periodic paralysis. Nature. 1991 Dec 5; 354(6352):387-9.
          View in: PubMed
        238. Cannon SC, Brown RH, Corey DP. A sodium channel defect in hyperkalemic periodic paralysis: potassium-induced failure of inactivation. Neuron. 1991 Apr; 6(4):619-26.
          View in: PubMed
        239. Brown RH, Horvitz HR, Rouleau GA, McKenna-Yasek D, Beard C, Sapp P, Haines JL, Gusella JF, Figlewicz DA. Gene linkage in familial amyotrophic lateral sclerosis: a progress report. Adv Neurol. 1991; 56:215-26.
          View in: PubMed
        240. Sklar RM, Brown RH. Methylprednisolone increases dystrophin levels by inhibiting myotube death during myogenesis of normal human muscle in vitro. J Neurol Sci. 1991 Jan; 101(1):73-81.
          View in: PubMed
        241. Sklar RM, Beggs AH, Lev AA, Specht L, Shapiro F, Brown RH. Defective dystrophin in Duchenne and Becker dystrophy myotubes in cell culture. Neurology. 1990 Dec; 40(12):1854-8.
          View in: PubMed
        242. Hoffman EP, Kunkel LM, Brown RH. Proteolytic fragment or new gene product? Nature. 1988 Nov 17; 336(6196):210.
          View in: PubMed
        243. Brown RH, Hoffman EP. Molecular biology of Duchenne muscular dystrophy. Trends Neurosci. 1988 Nov; 11(11):480-4.
          View in: PubMed
        244. Hoffman EP, Fischbeck KH, Brown RH, Johnson M, Medori R, Loike JD, Harris JB, Waterston R, Brooke M, Specht L, et al. Characterization of dystrophin in muscle-biopsy specimens from patients with Duchenne's or Becker's muscular dystrophy. N Engl J Med. 1988 May 26; 318(21):1363-8.
          View in: PubMed
        245. Hoffman EP, Brown RH, Kunkel LM. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell. 1987 Dec 24; 51(6):919-28.
          View in: PubMed
        246. Lev AA, Feener CC, Kunkel LM, Brown RH. Expression of the Duchenne's muscular dystrophy gene in cultured muscle cells. J Biol Chem. 1987 Nov 25; 262(33):15817-20.
          View in: PubMed
        247. Brown RH, Johnson D, Ogonowski M, Weiner HL. Antineural antibodies in the serum of patients with amyotrophic lateral sclerosis. Neurology. 1987 Jan; 37(1):152-5.
          View in: PubMed
        248. Brown RH, Schweitzer JS, Audhya T, Goldstein G, Dichter MA. Immunoreactive thymopoietin in the mouse central nervous system. Brain Res. 1986 Sep 3; 381(2):237-43.
          View in: PubMed
        249. Brown RH, Schweitzer J, Dichter MA. Expression of the Thy-1 antigen in long-term cultures of embryonic mouse spinal cord. Brain Res. 1984 Mar 26; 296(1):87-91.
          View in: PubMed
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