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Prof Linda Greensmith

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Personal Profile

Name: Linda Greensmith Email: l.greensmith@ucl.ac.uk
Title: Prof Tel: 020 3448 4280
Department: IoN Motor Neurosci & Mov Disorders Fax:
Position: Professor of Neuroscience Address: UCL Institute of Neurology, Queen Square, London, WC1N 3BG
Research Domain: Basic Life Sciences, Experimental Medicine, Neuroscience, Personalised Medicine, Stem Cells & Regenerative Medicine (Frontier Disciplines) Web Page: Personal Web Page

Profile

Research Description

The research undertaken in our labs is focused on disorders that affect the neuromuscular system. In particular, our work is aimed at improving our understanding of the mechanisms involved in motor neuron degeneration and dysfunction in motor neuron diseases such as Amyotrophic Lateral Sclerosis (ALS) and Spinal Bulbar Muscular Atrophy (SBMA). We are also examining the pathological mechanisms underlying forms of peripheral neuropathy such as Charcot Marie Tooth Disease (CMT)/distal Hereditary Motor Neuropathy (dHMN). A key a pathological feature of these disorders is aberrant protein aggregation, and our interest in this pathological mechanism has led us to establish an active research programme investigating Inclusion Body Myositis (IBM), the most common muscle disorder affecting adults over the age of 50. Interestingly, mutations in the gene valosin containing protein (VCP) gene are a cause of both familial ALS as well as a hereditary form of IBM known as Inclusion body myopathy associated with Paget's disease and frontotemporal dementia (IBMPFD), linking IBM with ALS and FTD


We use a multidisciplinary approach in our experiments, examining motor neurons and muscle cells in culture (both primary cells and embroyonic stem cell-derived) as well as in animal models of neuromuscular disease. We also use a wide range of techniques from cellular and molecular biology to whole animal, in vivo systems physiology. This combination of techniques allows us to examine the molecular and cellular changes that take place in cells in culture under highly controllable conditions, which can help us to identify pathological mechanisms which we can then target with a range of potential therapeutic agents or approaches, before moving on to test the validity of our findings in vivo using appropriate animal models. The overall aim of our research program is to help in the development of effective therapeutic strategies for use in the treatment of these debilitating and often fatal neurodegenerative and neuromuscular disorders.


Research Activities

Motor Neuron and Neuromuscular Disorders

Neuromuscular and Neurodegenerative Disorders

Neuronal chaperones and proteasomal sorting in Motor Neuron Disease

Protein biomarker

The neurogenetics of neurodegenerative disease

motor neuron disease

peripheral nerve disease especially inherited neuropathies but also inflammatory neuropathies

Education Description

UCL Collaborators

Prof Alan Thompson; Prof Tarek Yousry; Prof John Hardy; Dr Richard Orrell; Prof Jason Warren; Dr Axel Petzold; Prof Sebastian Brandner; Prof Elizabeth Fisher; Prof Michael Duchen; Dr Jeremy Garson; Prof Mary Reilly; Prof Nicholas Wood; Dr Shi-Yu Yang; Prof Martin Rossor; Prof Martin Koltzenburg; Prof Francesco Muntoni

External Collaborators

Publications

    2014

    • Caroll J, Page TK, Chiang SC, Kalmar B, Bode D, Greensmith L, McKinnon PJ, Thorpe JR, Hafezparast M, El-Khamisy SF (2014). Expression of a pathogenic mutation of SOD1 sensitises aprataxin deficient cells and mice to oxidative stress and triggers hallmarks of premature ageing.. Hum Mol Genet, , - . doi:10.1093/hmg/ddu500
    • Lu CH, Petzold A, Topping J, Allen K, Macdonald-Wallis C, Clarke J, Pearce N, Kuhle J, Giovannoni G, Fratta P, Sidle K, Fish M, Orrell R, Howard R, Greensmith L, Malaspina A (2014). Plasma neurofilament heavy chain levels and disease progression in amyotrophic lateral sclerosis: insights from a longitudinal study.. J Neurol Neurosurg Psychiatry, , - . doi:10.1136/jnnp-2014-307672
    • Petchey LK, Risebro CA, Vieira JM, Roberts T, Bryson JB, Greensmith L, Lythgoe MF, Riley PR (2014). Loss of Prox1 in striated muscle causes slow to fast skeletal muscle fiber conversion and dilated cardiomyopathy.. Proc Natl Acad Sci U S A, , - . doi:10.1073/pnas.1406191111
    • Montague K, Malik B, Gray AL, La Spada AR, Hanna MG, Szabadkai G, Greensmith L (2014). Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy.. Brain, , - . doi:10.1093/brain/awu114
    • Ricketts T, McGoldrick P, Fratta P, de Oliveira HM, Kent R, Phatak V, Brandner S, Blanco G, Greensmith L, Acevedo-Arozena A, Fisher EM (2014). A Nonsense Mutation in Mouse Tardbp Affects TDP43 Alternative Splicing Activity and Causes Limb-Clasping and Body Tone Defects.. PLoS One, 9(1), e85962 - . doi:10.1371/journal.pone.0085962
    • Bros-Facer V, Krull D, Taylor A, Dick JR, Bates SA, Cleveland MS, Prinjha RK, Greensmith L (2014). Treatment with an antibody directed against Nogo-A delays disease progression in the SOD1G93A mouse model of Amyotrophic Lateral Sclerosis.. Hum Mol Genet, , - . doi:10.1093/hmg/ddu136
    • Bryson JB, Machado CB, Crossley M, Stevenson D, Bros-Facer V, Burrone J, Greensmith L, Lieberam I (2014). Optical control of muscle function by transplantation of stem cell-derived motor neurons in mice.. Science, 344(6179), 94 - 97. doi:10.1126/science.1248523
    • Parfitt DA, Aguila M, McCulley CH, Bevilacqua D, Mendes HF, Athanasiou D, Novoselov SS, Kanuga N, Munro PM, Coffey PJ, Kalmar B, Greensmith L, Cheetham ME (2014). The heat-shock response co-inducer arimoclomol protects against retinal degeneration in rhodopsin retinitis pigmentosa.. Cell Death Dis, 5, e1236 - . doi:10.1038/cddis.2014.214
    • Fratta P, Nirmalananthan N, Masset L, Skorupinska I, Collins T, Cortese A, Pemble S, Malaspina A, Fisher EM, Greensmith L, Hanna MG (2014). Correlation of clinical and molecular features in spinal bulbar muscular atrophy.. Neurology, , - . doi:10.1212/WNL.0000000000000507

    2013

    • Machado P, Miller A, Herbelin L, He J, Noel J, Wang Y, McVey AL, Pasnoor M, Gallagher P, Statland J, Brady S, Lu C-H, Kalmar B, Sethi H, Samandouras G, Holton J, Greensmith L, Barohn RJ, Hanna MG, Dimachkie MM (2013). SAFETY AND TOLERABILITY OF ARIMOCLOMOL IN PATIENTS WITH SPORADIC INCLUSION BODY MYOSITIS: A RANDOMISED, DOUBLE-BLIND, PLACEBO-CONTROLLED, PHASE IIA PROOF-OF-CONCEPT TRIAL. ANNALS OF THE RHEUMATIC DISEASES, 72, 164 - 164.
    • Cortese A, Plagnol V, Brady S, Simone R, Lashley T, Acevedo-Arozena A, de Silva R, Greensmith L, Holton J, Hanna MG, Fisher EM, Fratta P (2013). Widespread RNA metabolism impairment in sporadic inclusion body myositis TDP43-proteinopathy.. Neurobiol Aging, , - . doi:10.1016/j.neurobiolaging.2013.12.029
    • Oates EC, Rosser AM, Hafezparast M, Lek M, Scoto M, Greensmith L, Auer-Grumbach M, Schule R, Herrmann DN, Clarke NF, MacArthur DG, Zuechner S, Muntoni F, Reilly MM, North KN (2013). Mutations in a new dynein/dynactin adaptor gene cause Dominant Congenital Spinal Muscular Atrophy (DCSMA) and Hereditary Spastic Paraplegia (HSP). doi:10.1016/j.nmd.2013.06.560
    • Novoselov SS, Mustill WJ, Gray AL, Dick JR, Kanuga N, Kalmar B, Greensmith L, Cheetham ME (2013). Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis.. PLoS One, 8(8), e73944 - . doi:10.1371/journal.pone.0073944
    • Schiavo G, Greensmith L, Hafezparast M, Fisher EM (2013). Cytoplasmic dynein heavy chain: the servant of many masters.. Trends Neurosci, , - . doi:10.1016/j.tins.2013.08.001
    • Kalmar B, Kolaszynska AK, Rossor AM, Houlden H, Schiavo G, Reilly MM, Greensmith L (2013). AXONAL TRANSPORT DEFICITS AND INCREASED VULNERABILITY TO OXIDATIVE STRESS IN MUTANT HSPB1 (HSP27)-INDUCED DISTAL HEREDITARY MOTOR NEUROPATHIES.
    • Crul T, Toth N, Piotto S, Literati-Nagy P, Tory K, Haldimann P, Kalmar B, Greensmith L, Torok Z, Balogh G, Gombos I, Campana F, Concilio S, Gallyas F, Nagy G, Berente Z, Gungor B, Peter M, Glatz A, Hunya A, Literati-Nagy Z, Vigh L, Hoogstra-Berends F, Heeres A, Kuipers I, Loen L, Seerden JP, Zhang D, Meijering RA, Henning RH, Brundel BJ, Kampinga HH, Koranyi L, Szilvassy Z, Mandl J, Sumegi B, Febbraio MA, Horvath I, Hooper PL, Vigh L (2013). Hydroximic acid derivatives: pleiotropic HSP co-inducers restoring homeostasis and robustness.. Curr Pharm Des, 19(3), 309 - 346.
    • McGoldrick P, Joyce P, Fisher EMC, Greensmith L (2013). Rodent models of amyotrophic lateral sclerosis. Biochimica et Biophysica Acta: international journal of biochemistry and biophysics, , - .
    • Malik B, Nirmalananthan N, Gray AL, La Spada AR, Hanna MG, Greensmith L (2013). Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy.. Brain, 136(Pt 3), 926 - 943. doi:10.1093/brain/aws343
    • Sumner C, d'Ydewalle C, Wooley J, Cornblath D, Fawcett K, Gardiner A, Reilly M, Houlden H, Kalmar B, Greensmith L, Rossor A, Hernandez D, Singleton A, Baloh R, Gonzalez M, Züchner S, Stanescu H, Kleta R, Mankodi A, Boylan K, Harms M (2013). A Dominant Mutation in FBXO38 Causes Distal Spinal Muscular Atrophy with Calf Predominance. American Journal of Human Genetics, , - . doi:10.1016/j.ajhg.2013.10.006
    • Kalmar B, Lu CH, Greensmith L (2013). The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol.. Pharmacol Ther, , - . doi:10.1016/j.pharmthera.2013.08.003
    • Mitchell JC, McGoldrick P, Vance C, Hortobagyi T, Sreedharan J, Rogelj B, Tudor EL, Smith BN, Klasen C, Miller CC, Cooper JD, Greensmith L, Shaw CE (2013). Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.. Acta Neuropathol, 125(2), 273 - 288. doi:10.1007/s00401-012-1043-z
    • Oates EC, Rossor AM, Hafezparast M, Gonzalez M, Speziani F, MacArthur DG, Lek M, Cottenie E, Scoto M, Foley AR, Hurles M, Houlden H, Greensmith L, Auer-Grumbach M, Pieber TR, Strom TM, Schule R, Herrmann DN, Sowden JE, Acsadi G, Menezes MP, Clarke NF, Züchner S, UK10K , Muntoni F, North KN, Reilly MM (2013). Mutations in BICD2 cause dominant congenital spinal muscular atrophy and hereditary spastic paraplegia.. Am J Hum Genet, 92(6), 965 - 973. doi:10.1016/j.ajhg.2013.04.018

    2012

    • Jessen KR, Arthur-Farraj, PJ , Latouche, M , Wilton, DK , Quintes, S , Chabrol, E , Banerjee, A , Woodhoo, A , Jenkins, B , Rahman, M , Turmaine, M , Wicher, GK , Mitter, R , Greensmith, L , Behrens, A , Raivich, G , Mirsky, R (2012). c-Jun Reprograms Schwann Cells of Injured Nerves to Generate a Repair Cell Essential for Regeneration. Neuron, 75(4), 633 - 647. doi:10.1016/j.neuron.2012.06.021
    • Rossor AM, Kalmar B, Greensmith L, Reilly MM (2012). The distal hereditary motor neuropathies.. J Neurol Neurosurg Psychiatry, 83(1), 6 - 14. doi:10.1136/jnnp-2011-300952
    • Rossor AM, Davidson GL, Blake J, Polke JM, Murphy SM, Houlden H, Innes A, Kalmar B, Greensmith L, Reilly MM (2012). A novel p.Glu175X premature stop mutation in the C-terminal end of HSP27 is a cause of CMT2.. J Peripher Nerv Syst, 17(2), 201 - 205. doi:10.1111/j.1529-8027.2012.00400.x
    • Wang Y, He J, McVey A, Pasnoor M, Gallagher P, Herbelin L, Statland J, Miller A, Greensmith L, Machado P, Hanna M, Barohn R, Dimachkie M (2012). Twelve-Month Change of IBMFRS in the Arimocolomol Inclusion Body Myositis Pilot Study.
    • McGoldrick P, Joyce P, Acevedo-Arozena A, Fisher E, Greensmith L (2012). Investigating new mutant models of MND.
    • Gray A, Nirmalananthan N, Malik B, Dick J, Hanna M, Greensmith L (2012). Targeting the endogenous stress response in a mouse model of SBMA.
    • Ahmed M, Miller A, Hanna MG, Greensmith L (2012). Investigating the effects of pharmacological up-regulation of the heat shock response in an in-vitro model of sporadic inclusion body myositis.
    • Rossor A, Kalmar B, Gray A, Mustill W, Schiavo G, Cheetham ME, Reilly MM, Greensmith L, Novoselov S (2012). AN IN-VITRO STUDY OF DISTAL HEREDITARY MOTOR NEUROPATHY DUE TO HOMOZYGOUS HSJ1 MUTATIONS. doi:10.1136/jnnp-2011-301993.209
    • Miller A, Ahmed M, Hanna MG, Greensmith L (2012). DEVELOPING NEW THERAPEUTIC STRATEGIES FOR INCLUSION BODY MYOSITIS. doi:10.1136/jnnp-2011-301993.140
    • Wade A, Thomas C, Kalmar B, Terenzio M, Garin J, Greensmith L, Schiavo G (2012). Activated leukocyte cell adhesion molecule modulates neurotrophin signaling.. J Neurochem, 121(4), 575 - 586. doi:10.1111/j.1471-4159.2012.07658.x
    • Smith AS, Passey S, Greensmith L, Mudera V, Lewis MP (2012). Characterization and optimization of a simple, repeatable system for the long term in vitro culture of aligned myotubes in 3D.. J Cell Biochem, 113(3), 1044 - 1053. doi:10.1002/jcb.23437
    • Lu CH, Petzold A, Kalmar B, Dick J, Malaspina A, Greensmith L (2012). Plasma neurofilament heavy chain levels correlate to markers of late stage disease progression and treatment response in SOD1(G93A) mice that model ALS.. PLoS One, 7(7), e40998 - . doi:10.1371/journal.pone.0040998
    • Bryson JB, Hobbs C, Parsons MJ, Bosch KD, Pandraud A, Walsh FS, Doherty P, Greensmith L (2012). Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis.. Hum Mol Genet, 21(17), 3871 - 3882. doi:10.1093/hmg/dds215
    • Kalmar B, Edet-Amana E, Greensmith L (2012). Treatment with a coinducer of the heat shock response delays muscle denervation in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.. Amyotroph Lateral Scler, 13(4), 378 - 392. doi:10.3109/17482968.2012.660953
    • Rossor AM, Davidson G, Houlden HH, Kalmar B, Greensmith L, Reilly MM (2012). A novel p.glu175x premature stop mutation in the C-terminal end of HSP27 is a cause of CMT2.
    • Fratta P, Malik B, Gray A, La Spada A, Hanna MG, Fisher EMC, Greensmith L (2012). FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion. Neurobiology of Aging, , - .

    2011

    • Rossor AM, Kalmar B, Cheetham M, Schiavo G, Reilly MM, Greensmith L (2011). AN IN-VITRO STUDY OF DISTAL HEREDITARY MOTOR NEUROPATHY DUE TO HOMOZYGOUS HSJ1 MUTATIONS.
    • Kalmar B, Innes A, Rossor AM, Houlden H, Schiavo G, Reilly MM, Greensmith L (2011). ASSESSMENT OF FUNCTIONAL IMPAIRMENTS IN CELLULAR MODELS OF MUTANT HSPB1 INDUCED NEUROPATHIES.
    • Miller A, Ahmed M, Hanna MG, Greensmith L (2011). The effects of arimoclomol on pathological outcome measures of inclusion body myositis in vitro.
    • Machado P, Miller A, Parton M, Dewar L, Holton JL, Dimachkie M, Herbelir L, Greensmith L, Barohn R, Hanna MG (2011). A randomised, double-blinded, placebo-controlled pilot study assessing the safety and tolerability of Arimoclomol in sporadic inclusion body myositis (IBM).
    • Ahmed M, Miller A, Hanna MG, Greensmith L (2011). Investigating the effects of pharmacological up-regulation of the heat shock response on protein degradation pathways in an in-vitro model of sporadic inclusion body myositis.
    • Lu CH, Kalmar B, Malaspina A, Greensmith L, Petzold A (2011). A method to solubilise protein aggregates for immunoassay quantification which overcomes the neurofilament "hook" effect.. J Neurosci Methods, 195(2), 143 - 150. doi:10.1016/j.jneumeth.2010.11.026
    • Innes A, Kalmar B, Houlden H, Reilly MM, Greensmith L (2011). Characterisation of novel mutations within heat shock protein 27 causing motor axonopathies.
    • Malik B, Nirmalananthan N, Bilsland LG, La Spada AR, Hanna MG, Schiavo G, Gallo JM, Greensmith L (2011). Absence of disturbed axonal transport in spinal and bulbar muscular atrophy.. Hum Mol Genet, 20(9), 1776 - 1786. doi:10.1093/hmg/ddr061
    • Boërio D, Greensmith L, Bostock H (2011). A model of mouse motor nerve excitability and the effects of polarizing currents.. J Peripher Nerv Syst, 16(4), 322 - 333. doi:10.1111/j.1529-8027.2011.00364.x
    • Acevedo-Arozena A, Kalmar B, Essa S, Ricketts T, Joyce P, Kent R, Rowe C, Parker A, Gray A, Hafezparast M, Thorpe JR, Greensmith L, Fisher EM (2011). A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis.. Dis Model Mech, 4(5), 686 - 700. doi:10.1242/dmm.007237
    • Banks GT, Haas MA, Line S, Shepherd HL, Alqatari M, Stewart S, Rishal I, Philpott A, Kalmar B, Kuta A, Groves M, Parkinson N, Acevedo-Arozena A, Brandner S, Bannerman D, Greensmith L, Hafezparast M, Koltzenburg M, Deacon R, Fainzilber M, Fisher EM (2011). Behavioral and other phenotypes in a cytoplasmic Dynein light intermediate chain 1 mutant mouse.. J Neurosci, 31(14), 5483 - 5494. doi:10.1523/JNEUROSCI.5244-10.2011
    • Gray A, Malik B, Montague K, Dick J, Hanna MG, Greensmith L (2011). Investigating pathophysiology and therapeutic strategies in a mouse model of spinal and bulbar muscular atrophy (SBMA).

    2010

    • Lu C, Malaspina A, Orrell R, Kalmar B, Petzold A, Greensmith L (2010). PLASMA NEUROFILAMENT HEAVY CHAIN LEVELS AS A DISEASE BIOMARKER IN THE SOD1 MOUSE MODEL OF AMYOTROPHIC LATERAL SCLEROSIS. doi:10.1136/jnnp.2010.226340.179
    • Bilsland LG, Sahai E, Kelly G, Golding M, Greensmith L, Schiavo G (2010). Deficits in axonal transport precede ALS symptoms in vivo.. Proc Natl Acad Sci U S A, 107(47), 20523 - 20528. doi:10.1073/pnas.1006869107
    • Miller A, Ahmed M, Hanna MG, Greensmith L (2010). PONM17 Augmentation of the heat shock response in an in vitro model of sporadic inclusion body myositis.. J Neurol Neurosurg Psychiatry, 81(11), e64 - . doi:10.1136/jnnp.2010.226340.190
    • Wade A, Greensmith L, Schiavo G (2010). Investigating a novel role for the cell surface molecule ALCAM in neurotrophin signalling.
    • Ahmed M, Miller AD, Hanna MG, Greensmith L (2010). Heat shock protein induction as a therapeutic strategy for inclusion body myositis.
    • Miller AD, Ahmed M, Hanna MG, Greensmith L (2010). Augmentation of the heat shock response in an in vitro model of sporadic inclusion body myositis.
    • Boërio D, Kalmar B, Greensmith L, Bostock H (2010). Excitability properties of mouse motor axons in the mutant SOD1(G93A) model of amyotrophic lateral sclerosis.. Muscle Nerve, 41(6), 774 - 784. doi:10.1002/mus.21579
    • El-Kadi AM, Bros-Facer V, Deng W, Philpott A, Stoddart E, Banks G, Jackson GS, Fisher EM, Duchen MR, Greensmith L, Moore AL, Hafezparast M (2010). The legs at odd angles (Loa) mutation in cytoplasmic dynein ameliorates mitochondrial function in SOD1G93A mouse model for motor neuron disease.. J Biol Chem, 285(24), 18627 - 18639. doi:10.1074/jbc.M110.129320
    • Stevens JC, Chia R, Hendriks WT, Bros-Facer V, van Minnen J, Martin JE, Jackson GS, Greensmith L, Schiavo G, Fisher EM (2010). Modification of superoxide dismutase 1 (SOD1) properties by a GFP tag--implications for research into amyotrophic lateral sclerosis (ALS).. PLoS One, 5(3), e9541 - . doi:10.1371/journal.pone.0009541
    • Ludolph AC, Bendotti C, Blaugrund E, Chio A, Greensmith L, Loeffler JP, Mead R, Niessen HG, Petri S, Pradat PF, Robberecht W, Ruegg M, Schwalenstöcker B, Stiller D, van den Berg L, Vieira F, von Horsten S (2010). Guidelines for preclinical animal research in ALS/MND: A consensus meeting.. Amyotroph Lateral Scler, 11(1-2), 38 - 45. doi:10.3109/17482960903545334
    • Lu C, Petzold A, Kalmar B, Orrell R, Malaspina A, Greensmith L (2010). Plasma neurofilament heavy chain levels as a disease biomarker in SOD1 mice..
    • Lu C, Malaspina A, Orrell R, Kalmar B, Petzold A, Greensmith L (2010). PONM06 Plasma neurofilament heavy chain levels as a disease biomarker in the SOD1 mouse model of amyotrophic lateral sclerosis.. J Neurol Neurosurg Psychiatry, 81(11), e62 - . doi:10.1136/jnnp.2010.226340.179
    • McGoldrick P, Dick J, Ricketts T, Acevedo-Arozena A, Fisher E, Greensmith L (2010). Investigating novel mutant mouse models of motor neuron disease.
    • Innes A, Kalmar B, Houlden H, Reilly MM, Greensmith L (2010). Characterisation of novel mutations within HSP27 causing Charcot-Marie-Tooth disease 2F and distal hereditary motor neuropathy II.

    2009

    • Stevens JC, Bros-Facer V, Hendriks W, van Minnen J, Greensmith L, Martin JE, Fisher EMC (2009). HOW DOES A DYNEIN MUTATION SLOW MOTOR NEURONE DISEASE?. doi:10.1136/jnnp.2009.191759b
    • 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 PM, Fisher EMC (2009). An ENU-induced mutation in mouse glycyl-tRNA synthetase (GARS) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy.. Disease Models and Mechanisms, 2(7-8), 359 - 373. doi:10.1242/dmm.002527
    • Mackenzie FE, Romero R, Williams D, Hilton H, Hough T, Greensmith L, Ribchester RR, Blanco G (2009). The TRPP Channel PKD1L2 Is Linked with a Complex Neuromuscular Disease in the Mouse.
    • Philpott A, Bros V, Flenniken A, Schiavo G, Fisher E, Greensmith L, Hafezparast M (2009). Cellular and molecular characterization of abnormal rear leg (Arl) - a new mouse strain with a point mutation in cytoplasmic dynein.
    • Stevens JC, Bros-Facer V, Hendriks W, van Minnen J, Greensmith L, Martin JE, Schiavo G, Fisher EMC (2009). An Investigation of SOD1 Behaviour in Amyotrophic Lateral Sclerosis.
    • Mackenzie FE, Romero R, Williams D, Gillingwater T, Hilton H, Dick J, Riddoch-Contreras J, Wong F, Ireson L, Powles-Glover N, Riley G, Underhill P, Hough T, Arkell R, Greensmith L, Ribchester RR, Blanco G (2009). Upregulation of PKD1L2 provokes a complex neuromuscular disease in the mouse.. Human Molecular Genetics, 18(19), 3553 - 3566.
    • Riddoch-Contreras J, Yang SY, Dick JRT, Goldspink G, Orrell RW, Greensmith L (2009). Mechano-growth factor, an IGF-I splice variant, rescues motoneurons and improves muscle function in SOD1(G93A) mice. Experimental Neurology, 215(2), 281 - 289. doi:10.1016/j.expneurol.2008.10.014
    • Banks GT, Bros-Facer V, Williams HP, Chia R, Achilli F, Bryson JB, Greensmith L, Fisher EM (2009). Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1(G93A) motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice.. PLoS One, 4(7), e6218. - .
    • AlQatari M, Vastani N, Bros-Facer V, Groves M, Greensmith L, Fisher EM, Koltzenburg M (2009). A MUTATION OF THE CYTOPLASMIC DYNEIN HEAVY CHAIN GENE Dync1h1 CAUSES A SEVERE SENSORY NEUROPATHY.
    • Kalmar B, Greensmith L (2009). Induction of heat shock proteins for protection against oxidative stress. Advanced Drug Delivery Reviews, 61(4), 310 - 318. doi:10.1016/j.addr.2009.02.003
    • Kalmar B, Greensmith L (2009). Activation of the heat shock response in a primary cellular model of motoneuron neurodegeneration-evidence for neuroprotective and neurotoxic effects.. Cellular and Molecular Biology Letters, 14(2), 319 - 335. doi:10.2478/s11658-009-0002-8
    • Boërio D, Greensmith L, Bostock H (2009). Excitability properties of motor axons in the maturing mouse. Journal of the Peripheral Nervous System, 14(1), 45 - 53. doi:10.1111/j.1529-8027.2009.00205.x

    2008

    • 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(5), 1271 - 1283. doi:10.1111/j.1471-4159.2008.05699.x
    • Nirmalananthan N, Dick JRT, La Spada AR, Greensmith L, Hanna MG (2008). A co-inducer of the heat shock response ameliorates disease in a mouse model of SBMA. doi:10.1016/j.nmd.2008.06.140
    • Hakim W, Smith A, Kazmi B, Kalmann B, Solomon A, Salih V, Greensmith L, Mudera V, Lewis MP (2008). Muscle-nerve interactions in chimaeric in vitro 3D collagen models.
    • Ateh DD, Hussain IK, Mustafa AH, Price KM, Gulati R, Nickols CD, Bird MM, Greensmith L, Hafezparast M, Fisher EMC, Baker CS, Martin JE (2008). Dynein-dynactin complex subunits are differentially localized in brain and spinal cord, with selective involvement in pathological features of neurodegenerative disease. Neuropathology and Applied Neurobiology, 34(1), 88 - 94. doi:10.1111/j.1365-2990.2007.00924.x
    • Bilsland LG, Greensmith L (2008). The endocannabinoid system in amyotrophic lateral sclerosis. Current Pharmaceutical Design, 14(23), 2306 - 2316.
    • Boerio D, Greensmith L, Bostock H (2008). Changes in excitability of mouse motor axons with age and in a SOD1 model of amyotrophic lateral sclerosis.
    • Gnanapavan S, Kalmar B, Keir G, Petzold A, Giovanonni G, Greensmith L (2008). Plasma neurofilament levels as a biomarker of disease progression in the SOD1G93A mouse model of amyotrophic lateral sclerosis.
    • Kalmar B, Greensmith L (2008). Heat shock proteins as therapeutic targets in Amyotrophic Lateral Sclerosis.. In Asea AAA, Brown IR (Ed.), Heat shock proteins and the brain: implications for neurodegenerative diseases and neuroprotection. Section 1- Heat shock proteins and neurodegenerative diseases. (pp. - ). : Springer.
    • Kalmar B, Novoselov S, Gray A, Cheetham ME, Margulis B, Greensmith L (2008). Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1(G93A) mouse model of ALS.. Journal of Neurochemistry, 107(2), 339 - 350. doi:10.1111/j.1471-4159.2008.05595.x

    2007

    • Yip L, Kalmar B, Greensmith L (2007). Investigating the role of astrocytes in ALS. NEURON GLIA BIOL, 2, S63 - S63.
    • Nirmalananthan N, Dick JRT, Thomas PS, La Spada AR, Greensmith L, Hanna MG (2007). Physiological analysis of the phenotype of a YAC transgenic mouse model of Kennedy's disease.

    2006

    • Bilsland LG, Dick JRT, Pryce G, Petrosino S, Di Marzo V, Baker D, Greensmith L (2006). Increasing cannabinoid levels by pharmacological and genetic manipulation delay disease progression in SOD1 mice.. The FASEB Journal, 20(7), 1003 - 1005. doi:10.1096/fj.05-4743fje
    • Ateh DD, Hussain I, Mustafa A, Price KM, Nickols CD, Bird MM, Greensmith L, Schiavo G, Hafezparast M, Fisher EMC, Baker CS, Martin JE (2006). Subcellular functional specificity of dynein-dynactin complex subunits - normal distribution and disturbances in neurodegenerative disease.
    • Ateh DD, Hussain I, Mustafa A, Price KM, Nickols CD, Bird MM, Greensmith L, Schiavo G, Hafezparast M, Fisher EMC, Baker CS, Martin JE (2006). Normal subcellular distribution of dynein-dynactin complex subunits and disturbances in neurodegenerative disease.
    • Mackenzie F, Ribchester R, Gillingwater T, Greensmith L, Powles-Glover N, Gale R, Arkell R, Blanco G (2006). Genetic mapping of ostesy, a novel mouse mutant showing poly-innervation at the NMJ and muscle atrophy.

    2005

    • Nirmalananthan N, Greensmith L (2005). Amyotrophic lateral sclerosis: recent advances and future therapies. Current Opinion in Neurology, 18(6), 712 - 719.
    • Kalmar B, Kieran D, Greensmith L (2005). Molecular chaperones as therapeutic targets in amyotrophic lateral sclerosis. Biochemical Society Transactions, 33(4), 551 - 552.
    • Achilli F, Boyle S, Kieran D, Chia R, Hafezparast M, Martin JE, Schiavo G, Greensmith L, Bickmore W, Fisher EMC (2005). The SOD1 transgene in the G93A mouse model of amyotrophic lateral sclerosis lies on distal mouse chromosome 12. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 6(2), 111 - 114.
    • Sharp PS, Dick JR, Greensmith L (2005). The effect of peripheral nerve injury on disease progression in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Neuroscience, 130(4), 897 - 910.
    • Ralph GS, Radcliffe PA, Day DM, Carthy JM, Leroux MA, Lee DCP, Wong LF, Bilsland LG, Greensmith L, Kingsman SM, Mitrophanous KA, Mazarakis ND, Azzouz M (2005). Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model. Nature Medicine, 11(4), 429 - 433. doi:10.1038/nm1205
    • Riddoch-Contreras J, Yang SY, Goldspink G, Orrell RW, Greensmith L (2005). Treatment with mechano-growth factor, an IGF-1 splice variant, rescues motoneurons and delays disease progression in SOD1 G93A mice..
    • Riddoch-Contreras J, Yang SY, Goldspink G, Orrell RW, Greensmith L (2005). Treatment with mechano-growth factor, an IGF-1 splice variant, rescues motoneurons and delays disease progression in SOD1 G93A mice..
    • Kieran D, Hafezparast M, Bohnert S, Dick JR, Martin J, Schiavo G, Fisher EMC, Greensmith L (2005). A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice.. J Cell Biol, 169(4), 561 - 567. doi:10.1083/jcb.200501085

    2004

    • Kieran DM, Kalmar B, Dick JRT, Riddoch-Contreras J, Burnstock G, Greensmith L (2004). Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nature Medicine, 10(4), 402 - 405. doi:10.1038/nm1021
    • Dekkers J, Bayley P, Dick JR, Schwaller B, Berchtold MW, Greensmith L (2004). Over-expression of parvalbumin in transgenic mice rescues motoneurons from injury-induced cell death. Neuroscience, 123(2), 459 - 466.
    • Kieran D, Greensmith L (2004). Inhibition of calpains, by treatment with leupeptin, improves motoneuron survival and muscle function in models of motoneuron degeneration. Neuroscience, 125(2), 427 - 439.
    • Ralph S, Radcliffe PA, Bilsland L, Greensmith L, Mitrophanous KA, Mazarakis ND, Azzouz M (2004). Targeted ablation of mutant SOD1 in ALS models using lentiviral mediated delivery of interfering RNA.
    • Powles NS, Ribchester R, Gillingwater T, Greensmith L, Arkell R, Blanco G (2004). Ostesy, a novel neuromuscular mutant.

    2003

    • Kalmar B, Greensmith L, Malcangio M, McMahon SB, Csermely P, Burnstock G (2003). The effect of treatment with BRX-220, a co-inducer of heat shock proteins, on sensory fibers of the rat following peripheral nerve injury. Experimental Neurology, 184(2), 636 - 647.
    • Low HL, Nógrádi A, Vrbová G, Greensmith L (2003). Axotomized motoneurons can be rescued from cell death by peripheral nerve grafts: the effect of donor age. Journal of Neuropathology and Experimental Neurology, 62(1), 75 - 87.
    • Kalmar B, Dekkers J, Burnstock G, Greensmith L (2003). Treatment with hsp27 protects injured motoneurons from cell death induced by apoptotic stimuli in vitro.
    • Sharp PS, Dekkers J, Dick JR, Greensmith L (2003). Manipulating transmitter release at the neuromuscular junction of neonatal rats alters the expression of ChAT and GAP-43 in motoneurons. Developmental Brain Research, 146(1-2), 29 - 38.

    2002

    • Dekkers J, Greensmith L, Navarrete R (2002). Changes in the expression of parvalbumin immunoreactivity in the lumbar spinal cord of the rat following neonatal nerve injury. Developmental Neuroscience, 24(4), 283 - 293. doi:10.1159/000066742
    • Kalmar B, Burnstock G, Vrbová G, Greensmith L (2002). The effect of neonatal nerve injury on the expression of heat shock proteins in developing rat motoneurones. Journal of Neurotrauma, 19(5), 667 - 679.
    • Kalmar B, Burnstock G, Vrbová G, Urbanics R, Csermely P, Greensmith L (2002). Upregulation of heat shock proteins rescues motoneurones from axotomy-induced cell death in neonatal rats. Experimental Neurology, 176(1), 87 - 97.

    2001

    • Dekkers J, Waters J, Vrbová G, Greensmith L (2001). Treatment of the neuromuscular junction with 4-aminopyridine results in improved reinnervation following nerve injury in neonatal rats. Neuroscience, 103(1), 267 - 274.

    2000

    • Dekkers J, Beckley I, Vrbova G, Greensmith L (2000). The effect of blocking integrins at the neuromuscular function on reinnervation in adult rats. EUR J NEUROSCI, 12, 288 - 288.
    • White CM, Greensmith L, Vrbová G (2000). Repeated stimuli for axonal growth causes motoneuron death in adult rats: the effect of botulinum toxin followed by partial denervation. Neuroscience, 95(4), 1101 - 1109.
    • Maleszak K, Slawinska U, Greensmith L, Vrbova G (2000). Long term effects of premature locomotor activity on the mechanical properties of muscle. EUR J NEUROSCI, 12, 149 - 149.
    • Greensmith L, Ng P, Mohaghegh P, Vrbová G (2000). Reducing transmitter release from nerve terminals influences motoneuron survival in developing rats. Neuroscience, 97(2), 357 - 362.
    • Sharp PS, Dekkers J, Greensmith L (2000). Manipulating transmitter release from developing motor nerve terminals alters the normal pattern of expression of transmitter and growth related proteins in developing motoneurons. EUR J NEUROSCI, 12, 341 - 341.

    1999

    • Wong KCK, Meyer T, Harding DI, Dick JRT, Vrbová G, Greensmith L (1999). Integrins at the neuromuscular junction are important for motoneuron survival. European Journal of Neuroscience, 11(9), 3287 - 3292.
    • Vrbová G, Greensmith L, Nógrádi A (1999). Neurotrophic factors.. Trends Neurosci, 22(3), 108 - 109.
    • Harding DI, Greensmith L, Vrbova G (1999). Stabilizing neuromuscular contacts reduces motoneuron death caused by paralysis of muscles in neonatal rats. Neuroscience, 93(3), 1141 - 1146.
    • Harding DI, Greensmith L, Mason M, Anderson PN, Vrbova G (1999). Overexpression of GAP-43 induces prolonged sprouting and causes death of adult motoneurons. European Journal of Neuroscience, 11, 2237 - 2242.

    1998

    • Greensmith L, Harding DI, Meyer M, Vrbova G (1998). Mechanical activity is necessary for the normal development of rat soleus muscle. J PHYSIOL-LONDON, 511P, 37S - 38S.
    • Greensmith L, Harding DI, Meyer MP, Vrbová G (1998). Mechanical activity is necessary for the elimination of polyneuronal innervation of developing rat soleus muscles. Developmental Brain Research, 110, 131 - 134.
    • Harding DI, Greensmith L, Anderson PN, Vrbová G (1998). Motoneurones induced to sprout remain susceptible to nerve injury in adult transgenic mice overexpressing GAP-43.
    • Harding DI, Greensmith L, Anderson PN, Vrbová G (1998). Motoneurons innervating partially denervated rat hindlimb muscles remain susceptible to axotomy-induced cell death. Neuroscience, 86, 291 - 299.
    • Sanusi J, Greensmith L, Lowrie MB, Vrbová G (1998). Motoneurones that innervate the rat soleus muscle mature later than those to the tibialis anterior and extensor digitorum longus muscles. Developmental Neuroscience, 20, 546 - 551.
    • Harding DI, Greensmith L, Anderson PN, Vrbova G (1998). Adult motoneurones in transgenic mice that overexpress GAP-43 are susceptible to nerve injury-induced cell death. J PHYSIOL-LONDON, 511P, 143P - 143P.
    • Meintanis S, Matsas R, Greensmith L, Mirsky R, Jessen KR (1998). An in vivo method to study inhibitors and enhancers of myelination during development of the rat sciatic nerve.. EUR J NEUROSCI, 10, 340 - 340.

    1997

    • Harding DI, Greensmith L, Anderson PN, Vrbova G (1997). Uninjured motoneurones to partially denervated rat hindlimb muscles are susceptible to nerve injury during late postnatal development. J PHYSIOL-LONDON, 501P, P97 - P98.
    • Connold AL, Greensmith L, Tyc F, Vrbová G (1997). A simple method for the local delivery of various substances to the rat neuromuscular system. Brain Research Protocols, 1, 79 - 82.
    • Greensmith L, Hind A, Vrbová G (1997). Neonatal paralysis of the rat soleus muscle selectively affects motoneurones from more caudal segments of the spinal cord. Developmental Brain Research, 98, 281 - 286.
    • Greensmith L, Vrbová G (1997). Disturbances of neuromuscular interaction may contribute to muscle weakness in spinal muscular atrophy. Neuromuscular Disorders, 7, 1 - 4.
    • Greensmith L, Vrbová G (1997). Disturbances of neuromuscular interaction may contribute to muscle weakness in spinal muscular atrophy.. Neuromuscul Disord, 7(6-7), 369 - 372.

    1996

    • Greensmith L, Hind AH, Vrbová G (1996). Transient disruption of nerve-muscle interaction shortly after birth permanently alters the development of the rat soleus muscle.. Developmental Brain Research, 94(2), 152 - 158.
    • Harding DI, Greensmith L, Connold AL, Vrbová G (1996). Stabilizing neuromuscular contacts increases motoneuron survival after neonatal nerve injury in rats.. Neuroscience, 70(3), 799 - 805. doi:10.1016/S0306-4522(96)83016-8
    • Greensmith L, Vrbová G (1996). Motoneurone survival: A functional approach.. Trends in Neurosciences, 19(11), 450 - 455. doi:10.1016/S0166-2236(96)20034-7
    • Greensmith L, Dick J, Emanuel AO, Vrbová G (1996). Induction of transmitter release at the neuromuscular junction prevents motoneuron death after axotomy in neonatal rats.. Neuroscience, 71(1), 213 - 220. doi:10.1016/0306-4522(95)00446-7

    1995

    • SANUSI J, GREENSMITH L, LOWRIE MB, VRBOVA G (1995). INCREASED LOCOMOTOR-ACTIVITY IN NEWBORN RATS CAUSES MOTONEURON DEATH. J PHYSIOL-LONDON, 483P, P40 - P40.
    • Greensmith L, Sanusi J, Mentis GZ, Vrbová G (1995). Transient muscle paralysis in neonatal rats renders motoneurons susceptible to N-methyl-D-aspartate-induced neurotoxicity.. Neuroscience, 64(1), 109 - 115.
    • Kerai B, Greensmith L, Vrbová G, Navarrete R (1995). Effect of transient neonatal muscle paralysis on the growth of soleus motoneurones in the rat. Developmental Brain Research, 85(1), 89 - 95. doi:10.1016/0165-3806(94)00198-9
    • EMANUEL AO, GREENSMITH L, VRBOVA G (1995). INDUCTION OF TRANSMITTER RELEASE AT THE MUSCULAR JUNCTION PREVENTS MOTONEURON AFTER AXOTOMY IN NEONATAL RATS. J PHYSIOL-LONDON, 487P, P46 - P47.
    • Greensmith L, Vrbová G (1995). Possible strategies for treatment of SMA patients: A neurobiologist's view.. Neuromuscular Disorders, 5(5), 359 - 369. doi:10.1016/0960-8966(94)00090-V
    • Dick J, Greensmith L, Vrbová G (1995). Blocking of NMDA receptors during a critical stage of development reduces the effects of nerve injury at birth on muscles and motoneurones. Neuromuscular Disorders, 5(5), 371 - 382. doi:10.1016/0960-8966(94)00088-Q
    • Greensmith L, Mooney EC, Waters HJ, Houlihan-Burne DG, Lowrie MB (1995). Magnesium ions reduce motoneuron death following nerve injury or exposure to N-methyl-D-aspartate in the developing rat.. Neuroscience, 68(3), 807 - 812.

    1994

    • MOONEY EC, GREENSMITH L, LOWRIE MB (1994). MAGNESIUM PROTECTS INJURED MOTONEURONS AGAINST NMDA-INDUCED NEUROTOXICITY IN THE RAT. J PHYSIOL-LONDON, 475P, P152 - P153.
    • Greensmith L, Mentis GZ, Vrbová G (1994). Blockade of N-methyl-D-aspartate receptors by MK-801 (dizocilpine maleate) rescues motoneurones in developing rats.. Developmental Brain Research, 81(2), 162 - 170.
    • Greensmith L, Hasan HI, Vrbová G (1994). Nerve injury increases the susceptibility of motoneurons to N-methyl-d-aspartate-induced neurotoxicity in the developing rat.. Neuroscience, 58(4), 727 - 733. doi:10.1016/0306-4522(94)90450-2
    • Greensmith L, Navarrete R (1994). Effect of neonatal nerve injury on the expression of Major Histocompatability Complex antigens in the rat spinal-cord.. Neurodegeneration, 3(3), 235 - 242.

    1993

    • KERAI B, GREENSMITH L, VRBOVA G, NAVARRETE R (1993). EFFECT OF TRANSIENT NEONATAL PARALYSIS ON SOLEUS MOTONEURON GROWTH IN THE RAT. J PHYSIOL-LONDON, 467, P111 - P111.
    • Mentis GZ, Greensmith L, Vrbová G (1993). Motoneurons destined to die are rescued by blocking n-methyl-d-aspartate receptors by MK-801.. Neuroscience, 54(2), 283 - 285. doi:10.1016/0306-4522(93)90253-C
    • GREENSMITH L, MENTIS GZ, VRBOYA G (1993). PREVENTION OF NERVE-MUSCLE INTERACTION IN NEONATAL RATS RENDERS MOTONEURONS SUSCEPTIBLE TO NMDA-INDUCED NEUROTOXICITY. J PHYSIOL-LONDON, 459, P165 - P165.
    • Greensmith L, Sieradzan K, Vrbová G (1993). Possible consequences of disruption of neuromuscular contacts in early development for motoneurone survival.. Acta Neurobiologiae Experimentalis, 53(1), 319 - 324.

    1992

    • Greensmith L, Vrbová G (1992). Alterations of nerve-muscle interaction during postnatal development influence motoneurone survival in rats.. Developmental Brain Research, 69(1), 125 - 131.
    • Vrbová G, Greensmith L, Sieradzan K (1992). Motor neuron disease model.. Nature, 360(6401), 216 - . doi:10.1038/360216b0

    1991

    • Greensmith L, Vrbová G (1991). Neuromuscular contacts in the developing rat soleus depend on muscle activity.. Developmental Brain Research, 62(1), 121 - 129.
    • GREENSMITH L, HASAN HI, VRBOVA G (1991). NERVE INJURY IN ANESTHETIZED NEONATAL RATS INCREASES THE SUBSEQUENT SUSCEPTIBILITY OF MOTONEURONS TO NMDA-INDUCED NEUROTOXICITY. J PHYSIOL-LONDON, 434, P20 - P20.

    1990

    • GREENSMITH L, VRBOVA G (1990). REGULATION OF CHOLINE-ACETYLTRANSFERASE IN DEVELOPING RAT MOTONEURONS. J PHYSIOL-LONDON, 426, P59 - P59.

    1989

    • GREENSMITH L, VRBOVA G (1989). EFFECT OF MUSCLE PARALYSIS ON MOTONEURON SURVIVAL IN NEONATAL RATS. J PHYSIOL-LONDON, 417, P148 - P148.

    1988

    • GREENSMITH L, LEHOUELLEUR J, VRBOVA G (1988). THE EFFECT OF EARLY MUSCLE PARALYSIS ON THE DISTRIBUTION OF INNERVATION IN THE NEWBORN RAT. J PHYSIOL-LONDON, 401, P52 - P52.

    1987

    • LEHOUELLEUR J, GREENSMITH L, VRBOVA G (1987). EFFECT OF ALPHA-BUNGAROTOXIN ON NEONATAL MURINE MOTOR-INNERVATION DEVELOPMENT. J PHYSIOL-PARIS, 82(3), A49 - A49.

    • Joyce P, Fratta P, Phatak V, McGoldrick P, Greensmith L, Fisher EMC, Acevedo-Arozena A (). A new mouse model of ALS carrying a point mutation in the mouse Sod1 gene..
    • McGoldrick P, Dick J, Ricketts T, Acevedo-Arozena A, Fisher E, Greensmith L (). Investigating novel mutant mouse models of motor neuron disease..