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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 - Graduate School of Biomedical Sciences
      DepartmentTranslational Science

      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. Kiskinis E, Sandoe J, Williams LA, Boulting GL, Moccia R, Wainger BJ, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle FT, Davis-Dusenbery BN, Ziller M, Oakley D, Ichida J, Di Costanzo S, Atwater N, Maeder ML, Goodwin MJ, Nemesh J, Handsaker RE, Paull D, Noggle S, McCarroll SA, Joung JK, Woolf CJ, Brown RH, Eggan K. Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1. Cell Stem Cell. 2014 Jun 5; 14(6):781-95.
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        2. 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.
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        3. Goris A, van Setten J, Diekstra F, Ripke S, Patsopoulos NA, Sawcer SJ. No evidence for shared genetic basis of common variants in multiple sclerosis and amyotrophic lateral sclerosis. Hum Mol Genet. 2014 Apr 1; 23(7):1916-22.
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        4. Takahashi Y, Fukuda Y, Yoshimura J, Toyoda A, Kurppa K, Moritoyo H, Belzil VV, Dion PA, Higasa K, Doi K, Ishiura H, Mitsui J, Date H, Ahsan B, Matsukawa T, Ichikawa Y, Moritoyo T, Ikoma M, Hashimoto T, Kimura F, Murayama S, Onodera O, Nishizawa M, Yoshida M, Atsuta N, Sobue G. ERBB4 Mutations that Disrupt the Neuregulin-ErbB4 Pathway Cause Amyotrophic Lateral Sclerosis Type 19. Am J Hum Genet. 2013 Nov 7; 93(5):900-5.
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        5. 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 1; 23(3):668-81.
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        6. Brown RH, Hauser SL. Introduction to a special edition of Annals: Therapeutic prospects. Ann Neurol. 2013 Sep; 74(3):A15.
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        7. Sreedharan J, Brown RH. Amyotrophic lateral sclerosis: Problems and prospects. Ann Neurol. 2013 Sep; 74(3):309-16.
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        8. 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.
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        9. 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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        10. 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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        11. 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.
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        12. 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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        13. 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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        14. 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.
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        15. 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 Dec; 48(3):391-8.
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        16. 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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        17. 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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        18. 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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        19. 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.
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        20. 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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        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 hSOD1G?³A 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. 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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        26. 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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        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. 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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        29. 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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        30. 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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        31. 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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        32. 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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        33. 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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        34. 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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        35. 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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        36. 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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        37. Brown RH. Medicine. A reinnervating microRNA. Science. 2009 Dec 11; 326(5959):1494-5.
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        38. 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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        39. 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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        40. 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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        41. 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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        42. 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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        43. 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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        44. 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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        45. 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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        46. 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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        47. 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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        48. 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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        49. 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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        50. 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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        51. 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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        52. 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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        53. Brown RH. Developmental biology. Neuron research leaps ahead. Science. 2008 Aug 29; 321(5893):1169-70.
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        54. 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.
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        55. 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.
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        56. 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.
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        57. 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.
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        58. 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.
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        59. 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.
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        60. 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.
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        61. 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.
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        62. 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.
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        63. 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.
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        64. Glover L, Brown RH. Dysferlin in membrane trafficking and patch repair. Traffic. 2007 Jul; 8(7):785-94.
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        65. 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.
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        66. 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.
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        67. 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.
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        68. 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.
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        69. 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.
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        70. 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.
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        71. Brown RH, Amato A. Calpainopathy and eosinophilic myositis. Ann Neurol. 2006 Jun; 59(6):875-7.
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        72. 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.
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        73. 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.
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        74. 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.
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        75. 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.
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        76. 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.
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        77. 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.
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        78. 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.
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        79. 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.
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        80. Brown RH. Amyotrophic lateral sclerosis--a new role for old drugs. N Engl J Med. 2005 Mar 31; 352(13):1376-8.
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        81. 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.
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        82. 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.
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        83. 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.
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        84. 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.
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        85. 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.
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        86. 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.
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        87. 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.
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        88. 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.
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        89. 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.
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        90. 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.
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        91. 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.
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        92. 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.
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        93. 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.
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        94. 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.
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        95. 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.
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        96. 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.
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        97. 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.
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        98. 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.
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        99. 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.
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        100. 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.
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        101. 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.
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        102. 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.
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        103. 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.
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        104. Brown RH, Robberecht W. Amyotrophic lateral sclerosis: pathogenesis. Semin Neurol. 2001 Jun; 21(2):131-9.
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        105. 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.
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        106. 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.
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        107. 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.
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        108. 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.
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        109. 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.
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        110. 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.
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        111. 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.
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        112. 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.
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        113. 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.
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        114. Brown RH. SOD1 aggregates in ALS: cause, correlate or consequence? Nat Med. 1998 Dec; 4(12):1362-4.
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        115. 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.
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        116. 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.
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        117. 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.
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        118. 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.
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        119. 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.
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        120. 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.
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        121. 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.
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        122. 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.
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        123. 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.
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        124. 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.
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        125. 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.
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        126. Brown RH. Dystrophin-associated proteins and the muscular dystrophies. Annu Rev Med. 1997; 48:457-66.
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        127. Brown RH, Miller JB. Progress, problems, and prospects for gene therapy in muscle. Curr Opin Rheumatol. 1996 Nov; 8(6):539-43.
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        128. 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.
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        129. 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.
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        130. Cudkowicz ME, Brown RH. An update on superoxide dismutase 1 in familial amyotrophic lateral sclerosis. J Neurol Sci. 1996 Aug; 139 Suppl:10-5.
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        131. 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.
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        132. 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.
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        133. Brown RH. Superoxide dismutase and familial amyotrophic lateral sclerosis: new insights into mechanisms and treatments. Ann Neurol. 1996 Feb; 39(2):145-6.
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        134. Brown RH. Dystrophin-associated proteins and the muscular dystrophies: a glossary. Brain Pathol. 1996 Jan; 6(1):19-24.
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        135. 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.
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        136. 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.
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        137. Brown RH. Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function. Curr Opin Neurobiol. 1995 Dec; 5(6):841-6.
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        138. 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.
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        139. 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.
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        140. 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.
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        141. Brown RH. Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice. Cell. 1995 Mar 10; 80(5):687-92.
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        142. 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.
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        143. 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.
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        144. Hosler BA, Brown RH. Copper/zinc superoxide dismutase mutations and free radical damage in amyotrophic lateral sclerosis. Adv Neurol. 1995; 68:41-6.
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        145. 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.
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        146. Brown RH. A transgenic-mouse model of amyotrophic lateral sclerosis. N Engl J Med. 1994 Oct 20; 331(16):1091-2.
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        147. 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.
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        148. 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.
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        149. 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.
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        150. 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.
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        151. 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.
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        152. Brown RH. Ion channel mutations in periodic paralysis and related myotonic diseases. Ann N Y Acad Sci. 1993 Dec 20; 707:305-16.
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        153. 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.
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        154. 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.
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        155. 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.
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        156. Rosen DR, Brown RH. Dinucleotide repeat polymorphism in the HOX4E locus. Hum Mol Genet. 1993 May; 2(5):617.
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        157. 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.
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        158. Hardiman O, Sklar RM, Brown RH. Methylprednisolone selectively affects dystrophin expression in human muscle cultures. Neurology. 1993 Feb; 43(2):342-5.
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        159. 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.
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        160. 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.
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        161. 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.
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        162. 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.
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        163. 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.
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        164. 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.
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        165. 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.
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