Robert H. Brown - Publications

Affiliations: 
Neurology University of Massachussetts Medical School, Worcester, MA, United States 

182 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2024 Weiss A, Gilbert JW, Flores IVR, Belgrad J, Ferguson C, Dogan EO, Wightman N, Mocarski K, Echeverria D, Summers A, Bramato B, McHugh N, Furgal R, Yamada N, Cooper D, ... ... Brown RH, et al. RNAi-mediated silencing of SOD1 profoundly extends survival and functional outcomes in ALS mice. Biorxiv : the Preprint Server For Biology. PMID 38979291 DOI: 10.1101/2024.06.20.599943  0.562
2023 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 Neuropathologica Communications. 11: 206. PMID 38124145 DOI: 10.1186/s40478-023-01709-4  0.515
2023 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 : a Journal of Neurology. PMID 37527465 DOI: 10.1093/brain/awad264  0.768
2022 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, ... ... Brown RH, et al. CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro. Nature Communications. 13: 6286. PMID 36271076 DOI: 10.1038/s41467-022-33332-7  0.552
2021 Johnson JO, Chia R, Miller DE, Li R, Kumaran R, Abramzon Y, Alahmady N, Renton AE, Topp SD, Gibbs JR, Cookson MR, Sabir MS, Dalgard CL, Troakes C, Jones AR, ... ... Brown RH, ... ... Brown RH, et al. Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis. Jama Neurology. PMID 34459874 DOI: 10.1001/jamaneurol.2021.2598  0.468
2021 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. Neurobiology of Disease. 105368. PMID 33892050 DOI: 10.1016/j.nbd.2021.105368  0.741
2020 Wainger BJ, Brown RH. Amyotrophic Lateral Sclerosis: Fuel for the Corticofugal Feud. Annals of Neurology. PMID 32741003 DOI: 10.1002/Ana.25861  0.329
2020 Mueller C, Berry JD, McKenna-Yasek DM, Gernoux G, Owegi MA, Pothier LM, Douthwright CL, Gelevski D, Luppino SD, Blackwood M, Wightman NS, Oakley DH, Frosch MP, Flotte TR, Cudkowicz ME, ... Brown RH, et al. Suppression with Adeno-Associated Virus and MicroRNA in Familial ALS. The New England Journal of Medicine. 383: 151-158. PMID 32640133 DOI: 10.1056/Nejmoa2005056  0.346
2020 Keeler AM, Zieger M, Semple C, Pucci L, Veinbachs A, Brown RH, Mueller C, ElMallah MK. Intralingual and Intrapleural AAV Gene Therapy Prolongs Survival in a SOD1 ALS Mouse Model. Molecular Therapy. Methods & Clinical Development. 17: 246-257. PMID 31970202 DOI: 10.1016/J.Omtm.2019.12.007  0.363
2019 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, ... ... Brown RH, et al. Human genetics and neuropathology suggest a link between miR-218 and amyotrophic lateral sclerosis pathophysiology. Science Translational Medicine. 11. PMID 31852800 DOI: 10.1126/Scitranslmed.Aav5264  0.485
2019 Giampetruzzi A, Danielson EW, Gumina V, Jeon M, Boopathy S, Brown RH, Ratti A, Landers JE, Fallini C. Modulation of actin polymerization affects nucleocytoplasmic transport in multiple forms of amyotrophic lateral sclerosis. Nature Communications. 10: 3827. PMID 31444357 DOI: 10.1038/S41467-019-11837-Y  0.338
2019 Gelfman S, Dugger SA, Araujo Martins Moreno C, Ren Z, Wolock CJ, Shneider N, Phatnani H, Cirulli ET, Lasseigne BN, Harris T, Maniatis T, Rouleau G, Brown RH, Gitler AD, Myers RM, et al. A new approach for rare variation collapsing on functional protein domains implicates specific genic regions in ALS. Genome Research. PMID 30940688 DOI: 10.1101/Gr.243592.118  0.322
2018 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. Science Translational Medicine. 10. PMID 30381409 DOI: 10.1126/Scitranslmed.Aau6414  0.37
2018 Medinas DB, Rozas P, Martínez 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. Proceedings of the National Academy of Sciences of the United States of America. PMID 30038021 DOI: 10.1073/Pnas.1801109115  0.306
2018 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. Experimental Neurology. PMID 30031021 DOI: 10.1016/J.Expneurol.2018.07.008  0.568
2018 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. Human Molecular Genetics. PMID 30010873 DOI: 10.1093/Hmg/Ddy260  0.772
2018 Bouley J, Chung DY, Ayata CY, Brown RH, Henninger N. Cortical spreading depression denotes concussion injury. Journal of Neurotrauma. PMID 29999455 DOI: 10.1089/Neu.2018.5844  0.53
2018 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, ... ... Brown RH, et al. Publisher Correction: TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD. Nature Neuroscience. PMID 29872124 DOI: 10.1038/S41593-018-0160-Y  0.695
2018 Al-Chalabi A, Brown RH. Finding a Treatment for ALS - Will Gene Editing Cut It? The New England Journal of Medicine. 378: 1454-1456. PMID 29641956 DOI: 10.1056/Nejmcibr1716741  0.365
2018 Nicolas A, Kenna KP, Renton AE, Ticozzi N, Faghri F, Chia R, Dominov JA, Kenna BJ, Nalls MA, Keagle P, Rivera AM, van Rheenen W, Murphy NA, van Vugt JJFA, Geiger JT, ... ... Brown RH, et al. Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron. 97: 1268-1283.e6. PMID 29566793 DOI: 10.1016/J.Neuron.2018.02.027  0.56
2018 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, ... ... Brown RH, et al. TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD. Nature Neuroscience. PMID 29556029 DOI: 10.1038/S41593-018-0113-5  0.746
2018 Fournier CN, Schoenfeld D, Berry JD, Cudkowicz ME, Chan J, Quinn C, Brown RH, Salameh JS, Tansey MG, Beers DR, Appel SH, Glass JD. An open label study of a novel immunosuppression intervention for the treatment of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 1-8. PMID 29308669 DOI: 10.1080/21678421.2017.1421666  0.319
2017 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. Molecular Neurodegeneration. 12: 46. PMID 28606110 DOI: 10.1186/S13024-017-0185-9  0.372
2017 Smith BN, Topp SD, Fallini C, Shibata H, Chen HJ, Troakes C, King A, Ticozzi N, Kenna KP, Soragia-Gkazi A, Miller JW, Sato A, Dias DM, Jeon M, Vance C, ... ... Brown RH, et al. Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis. Science Translational Medicine. 9. PMID 28469040 DOI: 10.1126/Scitranslmed.Aad9157  0.353
2017 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, ... ... Brown RH, et al. Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis. Science Translational Medicine. 9. PMID 28356511 DOI: 10.1126/Scitranslmed.Aai7866  0.302
2017 Ghasemi M, Brown RH. Genetics of Amyotrophic Lateral Sclerosis. Cold Spring Harbor Perspectives in Medicine. PMID 28270533 DOI: 10.1101/Cshperspect.A024125  0.361
2017 Brown RH, Schwartz WJ. David A. Drachman, MD (1932-2016). Neurology. 88: 928-929. PMID 28265038 DOI: 10.1212/Wnl.0000000000003757  0.507
2017 Czell D, Sapp PC, Neuwirth C, Weber M, Andersen PM, Brown RH. Further analysis of KIFAP3 gene in ALS patients from Switzerland and Sweden. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 1-3. PMID 28140676 DOI: 10.1080/21678421.2017.1280509  0.312
2016 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, Flint Beal M, Peters OM, ... ... Brown RH, et al. Mutant Profilin1 Transgenic Mice Recapitulate Cardinal Features of Motor Neuron Disease. Human Molecular Genetics. PMID 28040732 DOI: 10.1093/Hmg/Ddw429  0.749
2016 Taylor JP, Brown RH, Cleveland DW. Decoding ALS: from genes to mechanism. Nature. 539: 197-206. PMID 27830784 DOI: 10.1038/Nature20413  0.351
2016 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. Proceedings of the National Academy of Sciences of the United States of America. PMID 27681617 DOI: 10.1073/Pnas.1605964113  0.379
2016 van Zundert B, Brown RH. Silencing strategies for therapy of SOD1-mediated ALS. Neuroscience Letters. PMID 27507699 DOI: 10.1016/J.Neulet.2016.07.059  0.36
2016 van Rheenen W, Shatunov A, Dekker AM, McLaughlin RL, Diekstra FP, Pulit SL, van der Spek RA, Võsa U, de Jong S, Robinson MR, Yang J, Fogh I, van Doormaal PT, Tazelaar GH, Koppers M, ... ... Brown RH, et al. Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis. Nature Genetics. 48: 1043-8. PMID 27455348 DOI: 10.1038/Ng.3622  0.514
2016 Kenna KP, van Doormaal PT, Dekker AM, Ticozzi N, Kenna BJ, Diekstra FP, van Rheenen W, van Eijk KR, Jones AR, Keagle P, Shatunov A, Sproviero W, Smith BN, van Es MA, Topp SD, ... ... Brown RH, et al. NEK1 variants confer susceptibility to amyotrophic lateral sclerosis. Nature Genetics. PMID 27455347 DOI: 10.1038/Ng.3626  0.352
2016 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, ... ... Brown RH, et al. CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia. Nature Communications. 7: 11253. PMID 27080313 DOI: 10.1038/Ncomms11253  0.358
2016 Kubat Öktem E, Mruk K, Chang J, Akin A, Kobertz WR, Brown RH. Mutant SOD1 protein increases Nav1.3 channel excitability. Journal of Biological Physics. PMID 27072680 DOI: 10.1007/S10867-016-9411-X  0.353
2016 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 : a Journal of Neurology. PMID 26912636 DOI: 10.1093/Brain/Aww001  0.619
2016 Stoica L, Todeasa SH, Toro Cabrera G, Salameh JS, ElMallah MK, Mueller C, Brown RH, Sena-Esteves M. AAV delivered artificial microRNA extends survival and delays paralysis in an Amyotrophic Lateral Sclerosis mouse model. Annals of Neurology. PMID 26891182 DOI: 10.1002/Ana.24618  0.399
2016 Woehlbier U, Colombo A, Saaranen MJ, Pérez 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, ... ... Brown RH, et al. ALS-linked protein disulfide isomerase variants cause motor dysfunction. The Embo Journal. PMID 26869642 DOI: 10.15252/Embj.201592224  0.36
2016 A. Lu M, Rajanala S, Mikkilineni SV, Cahill CM, Brown R, D. Berry J, Rogers JT. The 5’-Untranslated Region of the C9orf72 mRNA Exhibits a Phylogenetic Alignment to the Cis-Aconitase Iron-Responsive Element; Novel Therapies for Amytrophic Lateral Sclerosis Neuroscience and Medicine. 7: 15-26. DOI: 10.4236/Nm.2016.71003  0.305
2016 Cabrera GT, Peters OM, Gendron TF, Gruntman A, Brown RH, Mueller C. 738. Engineered MicroRNA Silences C9ORF72 Variants in BAC Transgenic Mouse Molecular Therapy. 24. DOI: 10.1016/S1525-0016(16)33546-8  0.318
2015 Borel F, Gernoux G, Cardozo B, Metterville JP, Toro Cabrera G, Song L, Su Q, ElMallah MK, Brown R, Mueller C. Therapeutic rAAVrh.10-mediated SOD1 silencing in adult SOD1G93A mice and non-human primates. Human Gene Therapy. PMID 26710998 DOI: 10.1089/Hum.2015.122  0.368
2015 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, ... ... Brown RH, et al. Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice. Neuron. 88: 902-909. PMID 26637797 DOI: 10.1016/J.Neuron.2015.11.018  0.735
2015 Salameh JS, Brown RH, Berry JD. Amyotrophic Lateral Sclerosis: Review. Seminars in Neurology. 35: 469-76. PMID 26502769 DOI: 10.1055/S-0035-1558984  0.321
2015 Sreedharan J, Neukomm LJ, Brown RH, Freeman MR. Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2. Current Biology : Cb. PMID 26234214 DOI: 10.1016/J.Cub.2015.06.045  0.58
2015 Peters OM, Ghasemi M, Brown RH. Emerging mechanisms of molecular pathology in ALS. The Journal of Clinical Investigation. 125: 2548. PMID 26030230 DOI: 10.1172/JCI82693  0.699
2015 Peters OM, Ghasemi M, Brown RH. Emerging mechanisms of molecular pathology in ALS. The Journal of Clinical Investigation. 125: 1767-79. PMID 25932674 DOI: 10.1172/Jci71601  0.75
2015 Gonzalez-Perez P, Woehlbier U, Chian RJ, Sapp P, Rouleau GA, Leblond CS, Daoud H, Dion PA, Landers JE, Hetz C, Brown RH. Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients. Gene. 566: 158-65. PMID 25913742 DOI: 10.1016/J.Gene.2015.04.035  0.375
2015 Cirulli ET, Lasseigne BN, Petrovski S, Sapp PC, Dion PA, Leblond CS, Couthouis J, Lu YF, Wang Q, Krueger BJ, Ren Z, Keebler J, Han Y, Levy SE, Boone BE, ... ... Brown R, et al. Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways. Science (New York, N.Y.). 347: 1436-41. PMID 25700176 DOI: 10.1126/Science.Aaa3650  0.347
2015 Özo?uz A, Uyan Ö, Birdal G, Iskender C, Kartal E, Lahut S, Ömür Ö, Agim ZS, Eken AG, Sen NE, Kavak P, Sayg? C, Sapp PC, Keagle P, Parman Y, ... ... Brown RH, et al. The distinct genetic pattern of ALS in Turkey and novel mutations. Neurobiology of Aging. 36: 1764.e9-18. PMID 25681989 DOI: 10.1016/J.Neurobiolaging.2014.12.032  0.785
2015 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, et al. Novel mutations support a role for Profilin 1 in the pathogenesis of ALS. Neurobiology of Aging. 36: 1602.e17-27. PMID 25499087 DOI: 10.1016/J.Neurobiolaging.2014.10.032  0.373
2015 Stoica L, Salameh J, Mueller C, Brown R, Sena-Esteves M. 341. Therapeutic Approach for SOD1-ALS Using AAV9 Delivered Artificial microRNAs Molecular Therapy. 23. DOI: 10.1016/S1525-0016(16)33950-8  0.368
2014 Dominov JA, Uyan O, Sapp PC, McKenna-Yasek D, Nallamilli BR, Hegde M, Brown RH. A novel dysferlin mutant pseudoexon bypassed with antisense oligonucleotides. Annals of Clinical and Translational Neurology. 1: 703-20. PMID 25493284 DOI: 10.1002/Acn3.96  0.303
2014 Smith BN, Ticozzi N, Fallini C, Gkazi AS, Topp S, Kenna KP, Scotter EL, Kost J, Keagle P, Miller JW, Calini D, Vance C, Danielson EW, Troakes C, Tiloca C, ... ... Brown RH, et al. Exome-wide rare variant analysis identifies TUBA4A mutations associated with familial ALS. Neuron. 84: 324-31. PMID 25374358 DOI: 10.1016/J.Neuron.2014.09.027  0.339
2014 Diekstra FP, Van Deerlin VM, van Swieten JC, Al-Chalabi A, Ludolph AC, Weishaupt JH, Hardiman O, Landers JE, Brown RH, van Es MA, Pasterkamp RJ, Koppers M, Andersen PM, Estrada K, Rivadeneira F, et al. C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: a genome-wide meta-analysis. Annals of Neurology. 76: 120-33. PMID 24931836 DOI: 10.1002/Ana.24198  0.328
2014 Kiskinis E, Sandoe J, Williams LA, Boulting GL, Moccia R, Wainger BJ, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle FT, Davis-Dusenbery BN, Ziller M, Oakley D, ... ... Brown RH, et al. Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell. 14: 781-95. PMID 24704492 DOI: 10.1016/J.Stem.2014.03.004  0.364
2014 Wainger BJ, Kiskinis E, Mellin C, Wiskow O, Han SS, Sandoe J, Perez NP, Williams LA, Lee S, Boulting G, Berry JD, Brown RH, Cudkowicz ME, Bean BP, Eggan K, et al. Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons. Cell Reports. 7: 1-11. PMID 24703839 DOI: 10.1016/J.Celrep.2014.03.019  0.347
2014 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. Proceedings of the National Academy of Sciences of the United States of America. 111: E1121-9. PMID 24616503 DOI: 10.1073/Pnas.1322641111  0.378
2014 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. Proceedings of the National Academy of Sciences of the United States of America. 111: 1622-7. PMID 24474789 DOI: 10.1073/Pnas.1314826111  0.323
2014 Fogh I, Ratti A, Gellera C, Lin K, Tiloca C, Moskvina V, Corrado L, Sorarù G, Cereda C, Corti S, Gentilini D, Calini D, Castellotti B, Mazzini L, Querin G, ... ... Brown RH, et al. A genome-wide association meta-analysis identifies a novel locus at 17q11.2 associated with sporadic amyotrophic lateral sclerosis. Human Molecular Genetics. 23: 2220-31. PMID 24256812 DOI: 10.1093/Hmg/Ddt587  0.316
2014 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. Human Molecular Genetics. 23: 668-81. PMID 24108104 DOI: 10.1093/Hmg/Ddt454  0.369
2014 Kiskinis E, Sandoe J, Williams L, Boulting G, Moccia R, Wainger B, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle F, Davis-Dusenbery B, Ziller M, Oakley D, ... ... Brown R, et al. Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1 Cell Stem Cell. 14: 873. DOI: 10.1016/J.Stem.2014.04.005  0.339
2013 Takahashi Y, Fukuda Y, Yoshimura J, Toyoda A, Kurppa K, Moritoyo H, Belzil VV, Dion PA, Higasa K, Doi K, Ishiura H, Mitsui J, Date H, Ahsan B, Matsukawa T, ... ... Brown RH, et al. ERBB4 mutations that disrupt the neuregulin-ErbB4 pathway cause amyotrophic lateral sclerosis type 19. American Journal of Human Genetics. 93: 900-5. PMID 24119685 DOI: 10.1016/J.Ajhg.2013.09.008  0.362
2013 Sreedharan J, Brown RH. Amyotrophic lateral sclerosis: Problems and prospects. Annals of Neurology. 74: 309-16. PMID 24038380 DOI: 10.1002/Ana.24012  0.358
2013 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, ... ... Brown RH, et al. Inhibition of fast axonal transport by pathogenic SOD1 involves activation of p38 MAP kinase. Plos One. 8: e65235. PMID 23776455 DOI: 10.1371/Journal.Pone.0065235  0.301
2013 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. Human Molecular Genetics. 22: 3947-59. PMID 23736301 DOI: 10.1093/Hmg/Ddt249  0.331
2013 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. Journal of Neurophysiology. 109: 2803-14. PMID 23486205 DOI: 10.1152/Jn.00500.2012  0.325
2013 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. 16: 387-404. PMID 23143660 DOI: 10.1007/S10456-012-9322-9  0.365
2013 Smith BN, Newhouse S, Shatunov A, Vance C, Topp S, Johnson L, Miller J, Lee Y, Troakes C, Scott KM, Jones A, Gray I, Wright J, Hortobágyi T, Al-Sarraj S, ... ... Brown RH, et al. The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder. European Journal of Human Genetics : Ejhg. 21: 102-8. PMID 22692064 DOI: 10.1038/Ejhg.2012.98  0.352
2012 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, ... ... Brown RH, et al. Multimodal actions of neural stem cells in a mouse model of ALS: a meta-analysis. Science Translational Medicine. 4: 165ra164. PMID 23253611 DOI: 10.1126/Scitranslmed.3004579  0.344
2012 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. 79: 2201-8. PMID 23152587 DOI: 10.1212/Wnl.0B013E318275963B  0.333
2012 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, ... ... Brown RH, et al. EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Nature Medicine. 18: 1418-22. PMID 22922411 DOI: 10.1038/Nm.2901  0.373
2012 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, ... ... Brown RH, et al. Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature. 488: 499-503. PMID 22801503 DOI: 10.1038/Nature11280  0.373
2012 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. Neurobiology of Disease. 48: 391-8. PMID 22766032 DOI: 10.1016/J.Nbd.2012.06.018  0.349
2012 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, ... ... Brown RH, et al. dSarm/Sarm1 is required for activation of an injury-induced axon death pathway. Science (New York, N.Y.). 337: 481-4. PMID 22678360 DOI: 10.1126/Science.1223899  0.525
2012 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. Frontiers in Bioscience (Elite Edition). 4: 2801-8. PMID 22652679 DOI: 10.2741/E584  0.35
2012 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, ... ... Brown RH, et al. Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. Plos One. 7: e35333. PMID 22509407 DOI: 10.1371/Journal.Pone.0035333  0.348
2012 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. Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group On Motor Neuron Diseases. 13: 265-9. PMID 22409360 DOI: 10.3109/17482968.2011.653573  0.366
2012 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, et al. Polymorphisms in the GluR2 gene are not associated with amyotrophic lateral sclerosis. Neurobiology of Aging. 33: 418-20. PMID 20409611 DOI: 10.1016/J.Neurobiolaging.2010.03.007  0.341
2011 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, ... ... Brown RH, et al. Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Annals of Neurology. 70: 964-73. PMID 22190368 DOI: 10.1002/Ana.22611  0.305
2011 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 Biology. 9: e1001052. PMID 21541368 DOI: 10.1371/Journal.Pbio.1001052  0.33
2011 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. American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics : the Official Publication of the International Society of Psychiatric Genetics. 156: 285-90. PMID 21438137 DOI: 10.1002/Ajmg.B.31158  0.322
2011 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. Neurobiology of Aging. 32: 2096-9. PMID 20031275 DOI: 10.1016/J.Neurobiolaging.2009.11.018  0.308
2010 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. Nature Neuroscience. 13: 1396-403. PMID 20953194 DOI: 10.1038/Nn.2660  0.334
2010 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, ... ... Brown RH, et al. Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. The Lancet. Neurology. 9: 986-94. PMID 20801717 DOI: 10.1016/S1474-4422(10)70197-6  0.328
2010 Blauw HM, Al-Chalabi A, Andersen PM, van Vught PWJ, Diekstra FP, van Es MA, Saris CGJ, Groen EJN, van Rheenen W, Koppers M, Slot Rvt, Strengman E, Estrada K, Rivadeneira F, Hofman A, ... ... Brown RH, et al. A large genome scan for rare CNVs in amyotrophic lateral sclerosis Human Molecular Genetics. 19: 4091-4099. PMID 20685689 DOI: 10.1093/Hmg/Ddq323  0.339
2010 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. Neuroscience Letters. 482: 188-92. PMID 20638444 DOI: 10.1016/J.Neulet.2010.07.020  0.36
2010 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, et al. Paraoxonase gene mutations in amyotrophic lateral sclerosis. Annals of Neurology. 68: 102-7. PMID 20582942 DOI: 10.1002/Ana.21993  0.339
2010 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 Pathology (Zurich, Switzerland). 20: 1069-76. PMID 20579074 DOI: 10.1111/J.1750-3639.2010.00413.X  0.391
2010 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, et al. Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouse. Neurology. 74: 1687-93. PMID 20498436 DOI: 10.1212/Wnl.0B013E3181E042F7  0.341
2010 Collie AM, Landsverk ML, Ruzzo E, Mefford HC, Buysse K, Adkins JR, Knutzen DM, Barnett K, Brown RH, Parry GJ, Yum SW, Simpson DA, Olney RK, Chinnery PF, Eichler EE, et al. Non-recurrent SEPT9 duplications cause hereditary neuralgic amyotrophy. Journal of Medical Genetics. 47: 601-7. PMID 19939853 DOI: 10.1136/Jmg.2009.072348  0.3
2009 Brown RH. A Reinnervating MicroRNA Science. 326: 1494-1495. PMID 20007892 DOI: 10.1126/Science.1183842  0.329
2009 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. 4: e8175. PMID 19997636 DOI: 10.1371/Journal.Pone.0008175  0.313
2009 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 & Development. 23: 2294-306. PMID 19762508 DOI: 10.1101/Gad.1830709  0.344
2009 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. 73: 1180-1185. PMID 19741215 DOI: 10.1212/Wnl.0B013E3181Bbff05  0.306
2009 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. Neuroscience Letters. 463: 64-9. PMID 19596401 DOI: 10.1016/J.Neulet.2009.07.010  0.354
2009 Harel NY, Cudkowicz ME, Brown RH, Strittmatter SM. Serum Nogo-A levels are not elevated in amyotrophic lateral sclerosis patients. Biomarkers : Biochemical Indicators of Exposure, Response, and Susceptibility to Chemicals. 14: 414-7. PMID 19548774 DOI: 10.1080/13547500903056051  0.31
2009 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, ... ... Brown RH, et al. Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences of the United States of America. 106: 9004-9. PMID 19451621 DOI: 10.1073/Pnas.0812937106  0.326
2009 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, ... ... Brown RH, et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (New York, N.Y.). 323: 1205-8. PMID 19251627 DOI: 10.1126/Science.1166066  0.37
2009 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. American Journal of Human Genetics. 84: 85-8. PMID 19118816 DOI: 10.1016/J.Ajhg.2008.12.010  0.354
2009 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, ... ... Brown RH, et al. Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Human Molecular Genetics. 18: 472-81. PMID 18996918 DOI: 10.1093/Hmg/Ddn375  0.369
2008 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. Proceedings of the National Academy of Sciences of the United States of America. 105: 17913-8. PMID 18997009 DOI: 10.1073/Pnas.0804610105  0.354
2008 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. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 28: 10864-74. PMID 18945894 DOI: 10.1523/Jneurosci.1340-08.2008  0.339
2008 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, et al. A common haplotype within the PON1 promoter region is associated with sporadic ALS. Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group On Motor Neuron Diseases. 9: 306-14. PMID 18618303 DOI: 10.1080/17482960802233177  0.324
2008 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. Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group On Motor Neuron Diseases. 9: 141-8. PMID 18574757 DOI: 10.1080/17482960801934403  0.372
2008 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. 70: 929-34. PMID 18347314 DOI: 10.1212/01.Wnl.0000305956.37931.Dd  0.333
2008 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. 70: 278-283. PMID 18209202 DOI: 10.1212/01.Wnl.0000297552.13219.B4  0.315
2008 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 Journal of Neurology. 255: 99-102. PMID 18060566 DOI: 10.1007/S00415-007-0709-2  0.352
2008 Broom WJ, Johnson D, 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 Neuroscience Letters. 430: 241-245. PMID 18055113 DOI: 10.1016/J.Neulet.2007.11.004  0.347
2008 Robberecht W, Sapp P, Viaene MK, Rosen D, McKenna-Yasek D, Haines J, Horvitz R, Theys P, Brown R. Cu/Zn superoxide dismutase activity in familial and sporadic amyotrophic lateral sclerosis Journal of Neurochemistry. 62: 384-387. PMID 8263541 DOI: 10.1046/J.1471-4159.1994.62010384.X  0.356
2007 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, et al. Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery. Plos One. 2: e1254. PMID 18060051 DOI: 10.1371/Journal.Pone.0001254  0.334
2007 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. The Journal of Biological Chemistry. 282: 32480-90. PMID 17823119 DOI: 10.1074/Jbc.M704314200  0.326
2007 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. Journal of Neuropathology and Experimental Neurology. 66: 650-9. PMID 17620990 DOI: 10.1097/Nen.0B013E318093F40D  0.346
2006 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, et al. 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 Research. 1120: 1-12. PMID 17020749 DOI: 10.1016/J.Brainres.2006.08.079  0.32
2006 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) Amyotrophic Lateral Sclerosis. 7: 173-182. PMID 16963407 DOI: 10.1080/14660820600640596  0.319
2006 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. Journal of Biomolecular Screening. 11: 729-35. PMID 16928982 DOI: 10.1177/1087057106290937  0.371
2006 Pasinelli P, Brown RH. Molecular biology of amyotrophic lateral sclerosis: Insights from genetics Nature Reviews Neuroscience. 7: 710-723. PMID 16924260 DOI: 10.1038/Nrn1971  0.367
2006 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. 34: 603-7. PMID 16892429 DOI: 10.1002/Mus.20628  0.319
2006 Boston-Howes W, Gibb SL, Williams EO, Pasinelli P, Brown RH, Trotti D. Caspase-3 cleaves and inactivates the glutamate transporter EAAT2. The Journal of Biological Chemistry. 281: 14076-84. PMID 16567804 DOI: 10.1074/Jbc.M600653200  0.313
2006 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. Nature Genetics. 38: 411-3. PMID 16501576 DOI: 10.1038/Ng1742  0.38
2006 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. 66: 1218-22. PMID 16481598 DOI: 10.1212/01.Wnl.0000203129.82104.07  0.315
2006 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. Human Molecular Genetics. 15: 233-50. PMID 16321985 DOI: 10.1093/Hmg/Ddi440  0.339
2006 Broom WJ, Ay I, Pasinelli P, Brown RH. Inhibition of SOD1 expression by mitomycin C is a non-specific consequence of cellular toxicity Neuroscience Letters. 393: 184-188. PMID 16242241 DOI: 10.1016/J.Neulet.2005.09.064  0.37
2006 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. Neurobiology of Disease. 21: 102-9. PMID 16084104 DOI: 10.1016/J.Nbd.2005.06.016  0.392
2005 Rozen S, Cudkowicz ME, Bogdanov M, Matson WR, Kristal BS, Beecher C, Harrison S, Vouros P, Flarakos J, Vigneau-Callahan K, Matson TD, Newhall KM, Beal MF, Brown RH, Kaddurah-Daouk R. Metabolomic analysis and signatures in motor neuron disease. Metabolomics : Official Journal of the Metabolomic Society. 1: 101-108. PMID 18820733 DOI: 10.1007/S11306-005-4810-1  0.328
2005 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. Journal of Neurochemistry. 95: 1461-71. PMID 16313519 DOI: 10.1111/J.1471-4159.2005.03478.X  0.315
2005 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. Human Molecular Genetics. 14: 3507-21. PMID 16210380 DOI: 10.1093/Hmg/Ddi380  0.325
2005 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. Journal of Neurochemistry. 93: 1087-98. PMID 15934930 DOI: 10.1111/J.1471-4159.2005.03077.X  0.32
2005 Brown RH. Amyotrophic lateral sclerosis--a new role for old drugs. The New England Journal of Medicine. 352: 1376-1378. PMID 15800236 DOI: 10.1056/Nejmcibr050274  0.31
2005 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. Proceedings of the National Academy of Sciences of the United States of America. 102: 3639-44. PMID 15738401 DOI: 10.1073/Pnas.0408277102  0.308
2004 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. 5: 209-13. PMID 15657798 DOI: 10.1007/S10048-004-0193-0  0.312
2004 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. 43: 19-30. PMID 15233914 DOI: 10.1016/J.Neuron.2004.06.021  0.345
2004 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 Proceedings of the National Academy of Sciences of the United States of America. 101: 3178-3183. PMID 14981234 DOI: 10.1073/Pnas.0308726100  0.359
2004 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. Physiological Genomics. 16: 229-39. PMID 14645737 DOI: 10.1152/Physiolgenomics.00087.2001  0.353
2004 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 Research. 995: 84-96. PMID 14644474 DOI: 10.1016/J.Brainres.2003.09.063  0.302
2003 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 (New York, N.Y.). 302: 113-7. PMID 14526083 DOI: 10.1126/Science.1086071  0.345
2003 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. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group On Motor Neuron Diseases. 4: 62-73. PMID 14506936 DOI: 10.1080/14660820310011700  0.324
2003 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, et al. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurology. 61: 456-64. PMID 12939417 DOI: 10.1212/Wnl.61.4.456  0.311
2003 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, et al. Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis. American Journal of Human Genetics. 73: 397-403. PMID 12858291 DOI: 10.1086/377158  0.385
2003 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. Annals of Neurology. 54: 130-4. PMID 12838531 DOI: 10.1002/Ana.10638  0.377
2003 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. Nature Genetics. 33: 455-6. PMID 12627231 DOI: 10.1038/Ng1123  0.31
2002 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. 59: 729-734. PMID 12221165 DOI: 10.1212/Wnl.59.5.729  0.35
2002 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. 25: 122-123. PMID 11754197 DOI: 10.1002/Mus.1222  0.316
2002 Shefner JM, Cudkowicz ME, Brown RH. Comparison of incremental with multipoint MUNE methods in transgenic ALS mice. Muscle & Nerve. 25: 39-42. PMID 11754183 DOI: 10.1002/Mus.10000  0.301
2001 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. 57: 1857-61. PMID 11723276 DOI: 10.1212/Wnl.57.10.1857  0.352
2001 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. American Journal of Human Genetics. 70: 251-6. PMID 11706389 DOI: 10.1086/337945  0.36
2001 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, ... ... Brown RH, et al. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nature Genetics. 29: 166-73. PMID 11586298 DOI: 10.1038/Ng1001-166  0.353
2001 Brown RH, Robberecht W. Amyotrophic lateral sclerosis: pathogenesis. Seminars in Neurology. 21: 131-140. PMID 11442322 DOI: 10.1055/S-2001-15260  0.382
2001 Brown RH. Amyotrophic lateral sclerosis: lessons from mouse and human genetics Clinical Neuroscience Research. 1: 84-90. DOI: 10.1016/S1566-2772(00)00017-7  0.394
2001 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. Annals of Neurology. 49: 130-134. DOI: 10.1002/1531-8249(200101)49:1<130::Aid-Ana22>3.0.Co;2-0  0.303
2000 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 Radical Biology & Medicine. 29: 652-8. PMID 11033417 DOI: 10.1016/S0891-5849(00)00349-X  0.32
2000 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, et al. Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. Jama. 284: 1664-9. PMID 11015796 DOI: 10.1001/Jama.284.13.1664  0.313
1999 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. 53: 1239-46. PMID 10522879 DOI: 10.1212/Wnl.53.6.1239  0.32
1999 Parboosingh JS, Meininger V, McKenna-Yasek D, Brown RH, Rouleau GA. Deletions causing spinal muscular atrophy do not predispose to amyotrophic lateral sclerosis. Archives of Neurology. 56: 710-2. PMID 10369311 DOI: 10.1001/Archneur.56.6.710  0.373
1998 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. 1: 213-6. PMID 10737125 DOI: 10.1007/S100480050031  0.332
1998 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. Proceedings of the National Academy of Sciences of the United States of America. 95: 15763-8. PMID 9861044 DOI: 10.1073/Pnas.95.26.15763  0.352
1998 Brown RH. SOD1 aggregates in ALS: Cause, correlate or consequence? Nature Medicine. 4: 1362-1364. PMID 9846568 DOI: 10.1038/3945  0.364
1998 Browne SE, Bowling AC, Baik MJ, Gurney M, Brown RH, Beal MF. Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis. Journal of Neurochemistry. 71: 281-7. PMID 9648876 DOI: 10.1046/J.1471-4159.1998.71010281.X  0.353
1998 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. Annals of Neurology. 43: 703-10. PMID 9629839 DOI: 10.1002/Ana.410430604  0.335
1998 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. Annals of Neurology. 43: 645-53. PMID 9585360 DOI: 10.1002/Ana.410430514  0.326
1997 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. Journal of Neurochemistry. 69: 2064-74. PMID 9349552 DOI: 10.1046/J.1471-4159.1997.69052064.X  0.348
1997 Brown RH. Amyotrophic Lateral Sclerosis: Insights From Genetics Jama Neurology. 54: 1246-1250. PMID 9341570 DOI: 10.1001/Archneur.1997.00550220050013  0.342
1997 Friedlander RM, Brown RH, Gagliardini V, Wang J, Yuan J. Inhibition of ICE slows ALS in mice. Nature. 388: 31. PMID 9214497 DOI: 10.1038/40299  0.362
1997 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. Annals of Neurology. 41: 210-21. PMID 9029070 DOI: 10.1002/Ana.410410212  0.323
1996 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. 47: 1546-51. PMID 8960743 DOI: 10.1212/Wnl.47.6.1546  0.314
1996 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, et al. Three novel mutations and two variants in the gene for Cu/Zn superoxide dismutase in familial amyotrophic lateral sclerosis. Neuromuscular Disorders : Nmd. 6: 361-6. PMID 8938700 DOI: 10.1016/0960-8966(96)00353-7  0.362
1996 Cudkowicz ME, Brown RH. An update on superoxide dismutase 1 in familial amyotrophic lateral sclerosis. Journal of the Neurological Sciences. 139: 10-15. PMID 8899652 DOI: 10.1016/0022-510X(96)00084-6  0.365
1996 Vechio JD, Bruijn LI, Xu Z, Brown RH, Cleveland DW. Sequence variants in human neurofilament proteins: absence of linkage to familial amyotrophic lateral sclerosis. Annals of Neurology. 40: 603-10. PMID 8871580 DOI: 10.1002/Ana.410400410  0.362
1996 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 : a Journal For Neurodegenerative Disorders, Neuroprotection, and Neuroregeneration. 5: 57-64. PMID 8731383 DOI: 10.1006/Neur.1996.0008  0.333
1996 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. Nature Genetics. 13: 43-7. PMID 8673102 DOI: 10.1038/Ng0596-43  0.319
1995 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. Neuromuscular Disorders : Nmd. 5: 7-10. PMID 7719145 DOI: 10.1016/0960-8966(94)E0022-Z  0.327
1995 Mui S, Rebeck GW, McKenna-Yasek D, Hyman BT, Brown RH. Apolipoprotein E ε4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis Annals of Neurology. 38: 460-463. PMID 7668834 DOI: 10.1002/Ana.410380318  0.311
1995 Berger R, Mezey E, Clancy KP, Harta G, Wright RM, Repine JE, Brown RH, Brownstein M, Patterson D. Analysis of aldehyde oxidase and xanthine dehydrogenase/oxidase as possible candidate genes for autosomal recessive familial amyotrophic lateral sclerosis. Somatic Cell and Molecular Genetics. 21: 121-31. PMID 7570184 DOI: 10.1007/Bf02255787  0.344
1995 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. Neuromuscular Disorders : Nmd. 5: 353-7. PMID 7496169 DOI: 10.1016/0960-8966(95)00007-A  0.354
1994 Brown RH. A Transgenic-Mouse Model of Amyotrophic Lateral Sclerosis The New England Journal of Medicine. 331: 1091-1092. PMID 8090173 DOI: 10.1056/Nejm199410203311613  0.311
1994 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. American Journal of Medical Genetics. 51: 61-9. PMID 7913294 DOI: 10.1002/Ajmg.1320510114  0.325
1994 Rothstein JD, Bristol LA, Hosler B, Brown RH, Kuncl RW. Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons. Proceedings of the National Academy of Sciences of the United States of America. 91: 4155-9. PMID 7910402 DOI: 10.1073/Pnas.91.10.4155  0.314
1994 Figlewicz DA, McInnis MG, Goto J, Haines JL, Warren AC, Krizus A, Khodr N, Brown RH, McKenna-Yasek D, Antonarakis SE. Identification of flanking markers for the familial amyotrophic lateral sclerosis gene ALS1 on chromosome 21. Journal of the Neurological Sciences. 124: 90-5. PMID 7807155 DOI: 10.1016/0022-510X(94)90190-2  0.308
1993 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. Neuromuscular Disorders : Nmd. 3: 157-60. PMID 8358240 DOI: 10.1016/0960-8966(93)90008-8  0.34
1993 Bowling AC, Schulz JB, Brown RH, Beal MF. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis. Journal of Neurochemistry. 61: 2322-5. PMID 8245985 DOI: 10.1111/J.1471-4159.1993.Tb07478.X  0.31
1993 Brown RH. Mutations in Cu/Zn superoxide dismutase (SOD1) are associated with familial amyotrophic lateral sclerosis (ALS) Free Radical Biology and Medicine. 15: 471. DOI: 10.1016/0891-5849(93)90187-Y  0.309
1986 Brown RH, Hauser SL, Harrington H, Weiner HL. Failure of Immunosuppression With a Ten- to 14-Day Course of High-Dose Intravenous Cyclophosphamide to Alter the Progression of Amyotrophic Lateral Sclerosis Jama Neurology. 43: 383-384. PMID 3954622 DOI: 10.1001/Archneur.1986.00520040063021  0.329
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