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UCL Institute of Neurology

UCL Institute of Neurology
Queen Square, London WC1N 3BG UK

Prof. E. Fisher (Mammalian Molecular Genetics)
Tel: +44 207 676 2037
E-mail: e.fisher@prion.ucl.ac.uk

Prof. L. Greensmith (Pathophysiology of ALS)
Tel: +44 207 676 2161
E-mail: l.greensmith@ion.ucl.ac.uk

J. Hardy, PhD (Genetics of neurodegeneration)
Tel: +44 207 829 8722
E-mail: j.hardy@ion.ucl.ac.uk

ALS Clinic

The National Hospital for Neurology and Neurosurgery is one of only three MNDA care centres in London. This clinic covers a population of over 7 million, it sees over 200 patient episodes per year. The hospital is closely associated in its work with The UCL Institute of Neurology, and in combination they form a national and international centre at Queen Square for teaching, training and research in neurology and allied clinical and basic neurosciences.

Dr. R. Orrell (Clinical Neuroscience of ALS)
Tel: 020 7830 2387
E-mail: r.orrell@ucl.ac.uk

Research Lines

  • Mouse models of neurodegenerative disease including ALS
  • Cell biology of ALS
  • Development of therapeutic strategies for ALS
  • Genetics of neurodegeneration
  • ALS Biomarkers and MR imaging

Top 10 Publications

  1. Chiò A, Schymick JC, Restagno G, Scholz SW, Lombardo F, Lai SL, Mora G, Fung HC, Britton A, Arepalli S, Gibbs JR, Nalls M, Berger S, Kwee LC, Oddone EZ, Ding J, Crews C, Rafferty I, Washecka N, Hernandez D, Ferrucci L, Bandinelli S, Guralnik J, Macciardi F, Torri F, Lupoli S, Chanock SJ, Thomas G, Hunter DJ, Gieger C, Wichmann HE, Calvo A, Mutani R, Battistini S, Giannini F, Caponnetto C, Mancardi GL, La Bella V, Valentino F, Monsurrò MR, Tedeschi G, Marinou K, Sabatelli M, Conte A, Mandrioli J, Sola P, Salvi F, Bartolomei I, Siciliano G, Carlesi C, Orrell RW, Talbot K, Simmons Z, Connor J, Pioro EP, Dunkley T, Stephan DA, Kasperaviciute D, Fisher EM, Jabonka S, Sendtner M, Beck M, Bruijn L, Rothstein J, Schmidt S, Singleton A, Hardy J, Traynor BJ.
    A two-stage genome-wide association study of sporadic amyotrophic lateral sclerosis. Hum Mol Genet. 2009 Apr 15;18(8):1524-32. Epub 2009 Feb 4.
  2. Banks, G.T., Bros-Facer, V., Williams, H.P., Chia, R., Achilli, F., Bryson, J.B., Greensmith, L., Fisher, E.M.C. (2009) Mutant glycyl-tRNA synthetase ameliorates SOD1G93A motor neuron degeneration phenotype but has little effect on Loa dynein heavy chain mutant mice. PLoS ONE 4: e6218. PMID: 19593442
  3. Achilli, F, Bros-Facer, V, Williams, HP, Banks, GT, AlQatari, M, Chia, R, Tucci, V, Groves, M, Nickols, CD, Seburn, KL, Kendall, R, Cader, MZ, Talbot, K, van Minnen, J, Burgess, RW, Brandner, S, Martin, JE, Koltzenburg, M, Greensmith, L, Nolan PN, and Fisher EMC (2009) A novel mouse model with a point mutation in glycyl-tRNA synthetase (GARS) has axonal loss and profoundly reduced enzyme activity in homozygotes. Disease Models and Mechanisms 2 (7-8), 359-373.
  4. Bilsland LG, Nirmalananthan N, Yip J, Greensmith L & Duchen MR (2008) Expression of mutant SOD1G93A in astrocytes induces functional deficits in motoneuron mitochondria. Journal of Neurochemistry. 107, 1271-1283
  5. Schymick JC, Scholz SW, Fung HC, Britton A, Arepalli S, Gibbs JR, Lombardo F, Matarin M, Kasperaviciute D, Hernandez DG, Crews C, Bruijn L, Rothstein J, Mora G, Restagno G, Chiò A, Singleton A, Hardy J, Traynor BJ.
    Genome-wide genotyping in amyotrophic lateral sclerosis and neurologically normal controls: first stage analysis and public release of data. Lancet Neurol. 2007 Apr;6(4):322-8.
  6. Kasperaviciute, D., Weale, M.E., Shianna, K.V., Banks, G.T., Simpson, C.L., Hansen, V.K., Turner, M.R., Shaw, C.E., Al-Chalabi, A., Pall, H.S., Goodall, E.F., Morrison, K.E., Orrell, R.W., Beck, M., Jablonka, S., Sendtner, M., Brockington, A., Ince, P.G., Hartley, J., Nixon, H., Shaw, P.J., Schiavo, G., Wood, N.W., Goldstein, D.B., Fisher, E.M.C. (2007) Large-scale pathways-based association study in amyotrophic lateral sclerosis. Brain 130: 2292-2301 PMID: 17439985
  7. Schymick JC, Yang Y, Andersen PM, Vonsattel JP, Greenway M, Momeni P, Elder J, Chiò A, Restagno G, Robberecht W, Dahlberg C, Mukherjee O, Goate A, Graff-Radford N, Caselli RJ, Hutton M, Gass J, Cannon A, Rademakers R, Singleton AB, Hardiman O, Rothstein J, Hardy J, Traynor BJ. Progranulin mutations and amyotrophic lateral sclerosis or amyotrophic lateral sclerosis-frontotemporal dementia phenotypes. J Neurol Neurosurg Psychiatry. 2007 Jul;78(7):754-6. Epub 2007 Mar 19.
  8. Bilsland, LG, Dick, JRT, Pryce, G, Petrosino, S, Di Marzo, V, Baker D and Greensmith L(2006) The neuroprotective effects of cannabinoids in the SOD1G93A mouse model of ALS. FASEB Journal 20, 1003-1005.
  9. Kieran, DM, Hafezparast, M, Bohnert, S, Dick, JRT, Schaivo, G, Martin, J, Fisher EMC and Greensmith L (2005) Mutations in Dynein delay disease progression in SOD1(G93A) transgenic mice, a model of ALS. Journal of Cell Biology 169, 561-567
  10. Kieran, DM, Kalmar, B, Dick, JRT, Riddoch-Contreras, J, Burnstock G and Greensmith, L (2004). Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice Nature Medicine 10, 402-405.