Robert Brown to Animals
This is a "connection" page, showing publications Robert Brown has written about Animals.
Connection Strength
1.491
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Eichler FS, Brown RH. Gene therapy for neurologic disorders. Neurotherapeutics. 2024 Jul; 21(4):e00453.
Score: 0.065
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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.
Score: 0.056
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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 11; 29(11):1623-1630.
Score: 0.055
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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.
Score: 0.055
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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.
Score: 0.051
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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.
Score: 0.042
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van Zundert B, Brown RH. Silencing strategies for therapy of SOD1-mediated ALS. Neurosci Lett. 2017 01 01; 636:32-39.
Score: 0.038
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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.
Score: 0.036
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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.
Score: 0.036
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Brown RH, Al-Chalabi A. Endogenous retroviruses in ALS: A reawakening? Sci Transl Med. 2015 Sep 30; 7(307):307fs40.
Score: 0.035
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Peters OM, Ghasemi M, Brown RH. Emerging mechanisms of molecular pathology in ALS. J Clin Invest. 2015 May; 125(5):1767-79.
Score: 0.034
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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.
Score: 0.032
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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.
Score: 0.028
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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.
Score: 0.024
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Brown RH. Medicine. A reinnervating microRNA. Science. 2009 Dec 11; 326(5959):1494-5.
Score: 0.024
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Brown RH. Developmental biology. Neuron research leaps ahead. Science. 2008 Aug 29; 321(5893):1169-70.
Score: 0.022
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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.
Score: 0.021
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Glover L, Brown RH. Dysferlin in membrane trafficking and patch repair. Traffic. 2007 Jul; 8(7):785-94.
Score: 0.020
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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.
Score: 0.019
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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.
Score: 0.019
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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.
Score: 0.018
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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.
Score: 0.018
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Brown RH. Amyotrophic lateral sclerosis--a new role for old drugs. N Engl J Med. 2005 Mar 31; 352(13):1376-8.
Score: 0.017
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Dogan EO, Simonini SR, Bouley J, Weiss A, Brown RH, Henninger N. Genetic Ablation of Sarm1 Mitigates Disease Acceleration after Traumatic Brain Injury in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis. Ann Neurol. 2025 May; 97(5):963-975.
Score: 0.017
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Moazami MP, Rembetsy-Brown JM, Sarli SL, McEachern HR, Wang F, Ohara M, Wagh A, Kelly K, Krishnamurthy PM, Weiss A, Marosfoi M, King RM, Motwani M, Gray-Edwards H, Fitzgerald KA, Brown RH, Watts JK. Quantifying and mitigating motor phenotypes induced by antisense oligonucleotides in the central nervous system. Mol Ther. 2024 Dec 04; 32(12):4401-4417.
Score: 0.017
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Rivera Flores IV, Monopoli K, Jackson S, Echeverria D, O'Reilly D, Brown RH, Khvorova A. Near Sequence Homology Does Not Guarantee siRNA Cross-Species Efficacy. Nucleic Acid Ther. 2024 10; 34(5):234-244.
Score: 0.016
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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.
Score: 0.016
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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.
Score: 0.016
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Hossain MA, Sarin R, Donnelly DP, Miller BC, Weiss A, McAlary L, Antonyuk SV, Salisbury JP, Amin J, Conway JB, Watson SS, Winters JN, Xu Y, Alam N, Brahme RR, Shahbazian H, Sivasankar D, Padmakumar S, Sattarova A, Ponmudiyan AC, Gawde T, Verrill DE, Yang W, Kannapadi S, Plant LD, Auclair JR, Makowski L, Petsko GA, Ringe D, Agar NYR, Greenblatt DJ, Ondrechen MJ, Chen Y, Yerbury JJ, Manetsch R, Hasnain SS, Brown RH, Agar JN. Evaluating protein cross-linking as a therapeutic strategy to stabilize SOD1 variants in a mouse model of familial ALS. PLoS Biol. 2024 Jan; 22(1):e3002462.
Score: 0.016
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Yusuf IO, Parsi S, Ostrow LW, Brown RH, Thompson PR, Xu Z. PAD2 dysregulation and aberrant protein citrullination feature prominently in reactive astrogliosis and myelin protein aggregation in sporadic ALS. Neurobiol Dis. 2024 Mar; 192:106414.
Score: 0.016
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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.
Score: 0.016
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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 12 20; 11(1):206.
Score: 0.016
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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.
Score: 0.016
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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 03; 31(3-4):105-118.
Score: 0.015
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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.
Score: 0.015
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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).
Score: 0.015
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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).
Score: 0.015
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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.
Score: 0.014
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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.
Score: 0.014
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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.
Score: 0.014
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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.
Score: 0.014
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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.
Score: 0.014
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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.
Score: 0.013
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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.
Score: 0.013
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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).
Score: 0.012
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Watts JK, Brown RH, Khvorova A. Nucleic Acid Therapeutics for Neurological Diseases. Neurotherapeutics. 2019 04; 16(2):245-247.
Score: 0.011
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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.
Score: 0.011
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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).
Score: 0.011
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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.
Score: 0.011
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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.
Score: 0.011
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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.
Score: 0.011
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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.
Score: 0.011
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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.
Score: 0.010
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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).
Score: 0.010
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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.
Score: 0.010
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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.
Score: 0.010
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Taylor JP, Brown RH, Cleveland DW. Decoding ALS: from genes to mechanism. Nature. 2016 Nov 10; 539(7628):197-206.
Score: 0.010
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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.
Score: 0.010
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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.
Score: 0.009
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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.
Score: 0.009
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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.
Score: 0.009
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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.
Score: 0.009
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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.
Score: 0.009
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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.
Score: 0.008
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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.
Score: 0.008
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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.
Score: 0.008
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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.
Score: 0.008
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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.
Score: 0.008
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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.
Score: 0.008
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.007
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.006
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.005
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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.
Score: 0.004
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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.
Score: 0.004
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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.
Score: 0.004
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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.
Score: 0.004
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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.
Score: 0.004
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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.
Score: 0.004