Browse by UCL people
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Number of items: 79.
Article
Achilli, F;
Bros-Facer, V;
Williams, HP;
Banks, GT;
AlQatari, M;
Chia, R;
Tucci, V;
... Fisher, EMC; + view all
(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 & Mechanisms
, 2
(7-8)
359 - 373.
10.1242/dmm.002527.
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Ahmed, Mhoriam;
Spicer, Charlotte;
Harley, Jasmine;
Taylor, J Paul;
Hanna, Michael;
Patani, Rickie;
Greensmith, Linda;
(2023)
Amplifying the Heat Shock Response Ameliorates ALS and FTD Pathology in Mouse and Human Models.
Molecular Neurobiology
10.1007/s12035-023-03509-2.
(In press).
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Ahmed, M;
Machado, PM;
Miller, A;
Spicer, C;
Herbelin, L;
He, J;
Noel, J;
... Greensmith, L; + view all
(2016)
Targeting protein homeostasis in sporadic inclusion body myositis.
Science Translational Medicine
, 8
(331)
, Article 331ra41. 10.1126/scitranslmed.aad4583.
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Arthur-Farraj, PJ;
Latouche, M;
Wilton, DK;
Quintes, S;
Chabrol, E;
Banerjee, A;
Woodhoo, A;
... Jessen, KR; + view all
(2012)
c-Jun reprograms Schwann cells of injured nerves to generate a repair cell essential for regeneration.
Neuron
, 75
(4)
633 - 647.
10.1016/j.neuron.2012.06.021.
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Banks, G.T.;
Bros-Facer, V.;
Williams, H.P.;
Chia, R.;
Achilli, F.;
Bryson, J.B.;
Greensmith, L.;
(2009)
Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1G93A motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice.
PLoS ONE
, 4
(7)
, Article e6218. 10.1371/journal.pone.0006218.
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Banks, GT;
Bros-Facer, V;
Williams, HP;
Chia, R;
Achilli, F;
Bryson, JB;
Greensmith, L;
(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)
, Article e6218. 10.1371/journal.pone.0006218.
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Bendotti, C;
Bonetto, V;
Pupillo, E;
Logroscino, G;
Al-Chalabi, A;
Lunetta, C;
Riva, N;
... Beghi, E; + view all
(2020)
Focus on the heterogeneity of amyotrophic lateral sclerosis.
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
, 21
(7-8)
pp. 485-495.
10.1080/21678421.2020.1779298.
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Bennett, CL;
Dastidar, SG;
Ling, S-C;
Malik, B;
Ashe, T;
Wadhwa, M;
Miller, DB;
... La Spada, AR; + view all
(2018)
Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.
Acta Neuropathologica
, 136
(3)
pp. 425-443.
10.1007/s00401-018-1852-9.
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Bercsenyi, K;
Schmieg, N;
Bryson, JB;
Wallace, M;
Caccin, P;
Golding, M;
Zanotti, G;
... Schiavo, G; + view all
(2014)
Tetanus toxin entry. Nidogens are therapeutic targets for the prevention of tetanus.
Science
, 346
(6213)
1118 - 1123.
10.1126/science.1258138.
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Bondulich, MK;
Jolinon, N;
Osborne, GF;
Smith, EJ;
Rattray, I;
Neueder, A;
Sathasivam, K;
... Bates, GP; + view all
(2017)
Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice.
Scientific Reports
, 7
, Article 14275. 10.1038/s41598-017-14290-3.
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Bryson, JB;
Kourgiantaki, A;
Jiang, D;
Demosthenous, A;
Greensmith, L;
(2024)
An optogenetic cell therapy to restore control of target muscles in an aggressive mouse model of amyotrophic lateral sclerosis.
eLife
, 12
, Article RP88250. 10.7554/eLife.88250.
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Bryson, JB;
Barchellos Machado, C;
Lieberam, I;
Greensmith, L;
(2016)
Restoring motor function using optogenetics and neural engraftment.
Current Opinion in Biotechnology
, 40
pp. 75-81.
10.1016/j.copbio.2016.02.016.
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Bryson, JB;
Machado, CB;
Crossley, M;
Stevenson, D;
Bros-Facer, V;
Burrone, J;
Greensmith, L;
(2014)
Optical control of muscle function by transplantation of stem cell-derived motor neurons in mice.
Science
, 344
(6179)
94 - 97.
10.1126/science.1248523.
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Clark, AJ;
Kugathasan, U;
Baskozos, G;
Priestman, DA;
Fugger, N;
Lone, MA;
Othman, A;
... Bennett, DL; + view all
(2021)
An iPSC model of hereditary sensory neuropathy-1 reveals L-serine-responsive deficits in neuronal ganglioside composition and axoglial interactions.
Cell Reports Medicine
, 2
(7)
, Article 10034. 10.1016/j.xcrm.2021.100345.
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Clarke, BE;
Kalmar, B;
Greensmith, L;
(2022)
Enhanced Expression of TRAP1 Protects Mitochondrial Function in Motor Neurons under Conditions of Oxidative Stress.
International Journal of Molecular Sciences
, 23
(3)
, Article 1789. 10.3390/ijms23031789.
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Clarke, BE;
Gil, RS;
Yip, J;
Kalmar, B;
Greensmith, L;
(2019)
Regional differences in the inflammatory and heat shock response in glia: implications for ALS.
Cell Stress Chaperones
10.1007/s12192-019-01005-y.
(In press).
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Coenen-Stass, AML;
Magen, I;
Brooks, T;
Ben-Dov, IZ;
Greensmith, L;
Hornstein, E;
Fratta, P;
(2018)
Evaluation of Methodologies for Microrna Biomarker Detection by Next Generation Sequencing.
RNA Biology
, 15
(8)
pp. 1133-1145.
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Cortese, A;
Plagnol, V;
Brady, S;
Simone, R;
Lashley, T;
Acevedo-Arozena, A;
de Silva, R;
... Fratta, P; + view all
(2014)
Widespread RNA metabolism impairment in sporadic inclusion body myositis TDP43-proteinopathy.
Neurobiology of Aging
, 35
(6)
pp. 1491-1498.
10.1016/j.neurobiolaging.2013.12.029.
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Devoy, A;
Kalmar, B;
Stewart, M;
Park, H;
Burke, B;
Noy, SJ;
Redhead, Y;
... Fisher, EMC; + view all
(2017)
Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice.
Brain
, 140
(11)
pp. 2797-2805.
10.1093/brain/awx248.
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Fellows, AD;
Rhymes, ER;
Gibbs, KL;
Greensmith, L;
Schiavo, G;
(2020)
IGF1R regulates retrograde axonal transport of signalling endosomes in motor neurons.
EMBO Reports
, Article e49129. 10.15252/embr.201949129.
(In press).
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Forouhan, Mitra;
Lim, Wooi Fang;
Zanetti-Domingues, Laura C;
Tynan, Christopher J;
Roberts, Thomas C;
Malik, Bilal;
Manzano, Raquel;
... Rinaldi, Carlo; + view all
(2022)
AR cooperates with SMAD4 to maintain skeletal muscle homeostasis.
Acta Neuropathologica
, 143
(6)
pp. 713-731.
10.1007/s00401-022-02428-1.
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Fratta, P;
Malik, B;
Gray, A;
La Spada, AR;
Hanna, MG;
Fisher, EM;
Greensmith, L;
(2013)
FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion.
Neurobiology of Aging
, 34
(5)
1516.e17 - 1516.e19.
10.1016/j.neurobiolaging.2012.09.008.
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Fratta, P;
Nirmalananthan, N;
Masset, L;
Skorupinska, I;
Collins, T;
Cortese, A;
Pemble, S;
... Hanna, MG; + view all
(2014)
Correlation of clinical and molecular features in spinal bulbar muscular atrophy.
Neurology
, 82
(23)
pp. 2077-2084.
10.1212/WNL.0000000000000507.
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Fratta, P;
Sivakumar, P;
Humphrey, J;
Lo, K;
Ricketts, T;
Oliveira, H;
Brito-Armas, JM;
... Acevedo-Arozena, A; + view all
(2018)
Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
EMBO Journal
, 2018
, Article e98684. 10.15252/embj.201798684.
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Gibbs, KL;
Greensmith, L;
Schiavo, G;
(2015)
Regulation of Axonal Transport by Protein Kinases.
Trends in Biochemical Sciences
, 40
(10)
pp. 597-610.
10.1016/j.tibs.2015.08.003.
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Gibbs, KL;
Kalmar, B;
Rhymes, ER;
Fellows, AD;
Ahmed, M;
Whiting, P;
Davies, CH;
... Schiavo, G; + view all
(2018)
Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS.
Cell Death and Disease
, 9
, Article 596. 10.1038/s41419-018-0624-8.
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Gibbs, KL;
Kalmar, B;
Sleigh, JN;
Greensmith, L;
Schiavo, G;
(2016)
In vivo imaging of axonal transport in murine motor and sensory neurons.
Journal of Neuroscience Methods
, 257
pp. 26-33.
10.1016/j.jneumeth.2015.09.018.
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Gray, AL;
Annan, L;
Dick, JRT;
La Spada, AR;
Hanna, MG;
Greensmith, L;
Malik, B;
(2020)
Deterioration of muscle force and contractile characteristics are early pathological events in spinal and bulbar muscular atrophy mice.
Disease Models & Mechanisms
10.1242/dmm.042424.
(In press).
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Greensmith, Linda;
Bryson, J Barney;
(2023)
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1G93A mice.
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
10.1080/21678421.2023.2239867.
(In press).
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Greensmith, L;
Pradat, PF;
Sorarù, G;
Pennuto, M;
European SBMA Consortium;
(2019)
241st ENMC international workshop: Towards a European unifying lab for Kennedy's disease. 15–17th February, 2019 Hoofddorp, The Netherlands.
Neuromuscular Disorders
, 29
(9)
pp. 716-724.
10.1016/j.nmd.2019.07.008.
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Hagemann, C;
Tyzack, GE;
Taha, DM;
Devine, H;
Greensmith, L;
Newcombe, J;
Patani, R;
... Luisier, R; + view all
(2021)
Automated and unbiased discrimination of ALS from control tissue at single cell resolution.
Brain Pathology
, Article e12937. 10.1111/bpa.12937.
(In press).
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Joyce, PI;
Fratta, P;
Landman, AS;
Mcgoldrick, P;
Wackerhage, H;
Groves, M;
Busam, BS;
... Acevedo-Arozena, A; + view all
(2016)
Deficiency of the zinc finger protein ZFP106 causes motor and sensory neurodegeneration.
Human Molecular Genetics
, 25
(2)
pp. 291-307.
10.1093/hmg/ddv471.
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Kalmar, B;
Greensmith, L;
(2017)
Cellular Chaperones As Therapeutic Targets in ALS to Restore Protein Homeostasis and Improve Cellular Function.
Frontiers in Molecular Neuroscience
, 10
, Article 251. 10.3389/fnmol.2017.00251.
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Kalmar, B;
Innes, A;
Wanisch, K;
Koyen Kolaszynska, A;
Pandraud, A;
Kelly, G;
Abramov, AY;
... Greensmith, L; + view all
(2017)
Mitochondrial deficits and abnormal mitochondrial retrograde axonal transport play a role in the pathogenesis of mutant Hsp27 induced Charcot Marie Tooth Disease.
Human Molecular Genetics
, 26
(17)
pp. 3313-3326.
10.1093/hmg/ddx216.
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Kieran, D.;
Hafezparast, M.;
Bohnert, S.;
Dick, J.R.T.;
Martin, J.;
Schiavo, G.;
Fisher, E.M.C.;
(2005)
A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice.
The Journal of Cell Biology
, 169
(4)
pp. 561-567.
10.1083/jcb.200501085.
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Klickovic, U;
Zampedri, L;
Sinclair, CDJ;
Wastling, SJ;
Trimmel, K;
Howard, RS;
Malaspina, A;
... Fratta, P; + view all
(2019)
Skeletal muscle MRI differentiates SBMA and ALS and correlates with disease severity.
Neurology
10.1212/WNL.0000000000008009.
(In press).
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Liu, Fangqi;
Wu, Yu;
Almarri, Noora;
Habibollahi, Maryam;
Lancashire, Henry T;
Bryson, Barney;
Greensmith, Linda;
... Demosthenous, Andreas; + view all
(2022)
A Fully Implantable Opto-Electro Closed-Loop Neural Interface for Motor Neuron Disease Studies.
IEEE Transactions on Biomedical Circuits and Systems
10.1109/TBCAS.2022.3202026.
(In press).
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Lombardi, V;
Bombaci, A;
Zampedri, L;
Lu, C-H;
Malik, B;
Zetterberg, H;
Heslegrave, AJ;
... Fratta, P; + view all
(2019)
Plasma pNfH levels differentiate SBMA from ALS.
Journal of Neurology, Neurosurgery & Psychiatry
10.1136/jnnp-2019-320624.
(In press).
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Lombardi, V;
Querin, G;
Ziff, OJ;
Zampedri, L;
Martinelli, I;
Heller, C;
Foiani, M;
... Fratta, P; + view all
(2019)
Muscle and not neuronal biomarkers correlate with severity in spinal and bulbar muscular atrophy.
Neurology
, 92
(11)
10.1212/WNL.0000000000007097.
(In press).
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Lu, C-H;
Macdonald-Wallis, C;
Gray, E;
Pearce, N;
Petzold, A;
Norgren, N;
Giovannoni, G;
... Malaspina, A; + view all
(2015)
Neurofilament light chain A prognostic biomarker in amyotrophic lateral sclerosis.
Neurology
, 84
(22)
pp. 2247-2257.
10.1212/WNL.0000000000001642.
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Lu, CH;
Allen, K;
Oei, F;
Leoni, E;
Kuhle, J;
Tree, T;
Fratta, P;
... Malaspina, A; + view all
(2016)
Systemic inflammatory response and neuromuscular involvement in amyotrophic lateral sclerosis.
Neurology: Neuroimmunology & Neuroinflammation
, 3
(4)
, Article e244. 10.1212/NXI.0000000000000244.
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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)
, Article e40998. 10.1371/journal.pone.0040998.
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Lu, CH;
Petzold, A;
Topping, J;
Allen, K;
Macdonald-Wallis, C;
Clarke, J;
Pearce, N;
... Malaspina, A; + view all
(2015)
Plasma neurofilament heavy chain levels and disease progression in amyotrophic lateral sclerosis: insights from a longitudinal study.
Journal of Neurology, Neurosurgery & Psychiatry
, 86
(5)
pp. 565-573.
10.1136/jnnp-2014-307672.
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Luisier, R;
Tyzack, GE;
Hall, CE;
Mitchell, JS;
Devine, H;
Taha, DM;
Malik, B;
... Patani, R; + view all
(2018)
Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS.
Nature Communications
, 9
, Article 2010. 10.1038/s41467-018-04373-8.
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Machado, Pedro M;
McDermott, Michael P;
Blaettler, Thomas;
Sundgreen, Claus;
Amato, Anthony A;
Ciafaloni, Emma;
Freimer, Miriam;
... Arimoclomol in IBM Investigator Team of the Neuromuscular, Study; + view all
(2023)
Safety and efficacy of arimoclomol for inclusion body myositis: a multicentre, randomised, double-blind, placebo-controlled trial.
The Lancet Neurology
, 22
(10)
pp. 900-911.
10.1016/S1474-4422(23)00275-2.
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Machado, PM;
Ahmed, M;
Brady, S;
Gang, Q;
Healy, E;
Morrow, JM;
Wallace, AC;
... Hanna, MG; + view all
(2014)
Ongoing developments in sporadic inclusion body myositis.
Curr Rheumatol Rep
, 16
(12)
, Article 477. 10.1007/s11926-014-0477-9.
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Magen, I;
Yacovzada, NS;
Yanowski, E;
Coenen-Stass, A;
Grosskreutz, J;
Lu, C-H;
Greensmith, L;
... Hornstein, E; + view all
(2021)
Circulating miR-181 is a prognostic biomarker for amyotrophic lateral sclerosis.
Nature Neuroscience
, 24
(11)
pp. 1534-1541.
10.1038/s41593-021-00936-z.
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Malik, B;
Devine, H;
Patani, R;
La Spada, AR;
Hanna, MG;
Greensmith, L;
(2019)
Gene expression analysis reveals early dysregulation of disease pathways and links Chmp7 to pathogenesis of spinal and bulbar muscular atrophy.
Scientific Reports
, 9
, Article 3539. 10.1038/s41598-019-40118-3.
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Martin, NR;
Passey, SL;
Player, DJ;
Mudera, V;
Baar, K;
Greensmith, L;
Lewis, MP;
(2015)
Neuromuscular Junction Formation in Tissue-Engineered Skeletal Muscle Augments Contractile Function and Improves Cytoskeletal Organization.
Tissue Engineering Part A
, 21
(19-20)
pp. 2595-2604.
10.1089/ten.TEA.2015.0146.
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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
, 137
(7)
pp. 1894-1906.
10.1093/brain/awu114.
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Novoselov, SS;
Mustill, WJ;
Gray, AL;
Dick, JR;
Kanuga, N;
Kalmar, B;
Greensmith, L;
(2013)
Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis.
PLoS One
, 8
(8)
, Article e73944. 10.1371/journal.pone.0073944.
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Parfitt, DA;
Aguila, M;
McCulley, CH;
Bevilacqua, D;
Mendes, HF;
Athanasiou, D;
Novoselov, SS;
... Cheetham, ME; + view all
(2014)
The heat-shock response co-inducer arimoclomol protects against retinal degeneration in rhodopsin retinitis pigmentosa.
Cell Death Dis
, 5
, Article e1236. 10.1038/cddis.2014.214.
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Ricketts, T;
McGoldrick, P;
Fratta, P;
de Oliveira, HM;
Kent, R;
Phatak, V;
Brandner, S;
... Fisher, EM; + view all
(2014)
A nonsense mutation in mouse tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.
PLoS One
, 9
(1)
, Article e85962. 10.1371/journal.pone.0085962.
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Rinaldi, C;
Malik, B;
Greensmith, L;
(2016)
Targeted Molecular Therapies for SBMA.
Journal of Molecular Neuroscience
, 58
(3)
pp. 335-342.
10.1007/s12031-015-0676-5.
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Rossor, AM;
Liu, CH;
Petzold, A;
Malaspina, A;
Laura, M;
Greensmith, L;
Reilly, MM;
(2016)
Plasma neurofilament heavy chain is not a useful biomarker in Charcot–Marie–Tooth disease.
Muscle & Nerve
, 53
(6)
pp. 972-975.
10.1002/mus.25124.
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Rusmini, P;
Crippa, V;
Cristofani, R;
Rinaldi, C;
Cicardi, ME;
Galbiati, M;
Carra, S;
... Poletti, A; + view all
(2016)
The Role of the Protein Quality Control System in SBMA.
Journal of Molecular Neuroscience
, 58
(3)
pp. 348-364.
10.1007/s12031-015-0675-6.
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San Gil, R;
Clarke, BE;
Ecroyd, H;
Kalmar, B;
Greensmith, L;
(2021)
Regional Differences in Heat Shock Protein 25 Expression in Brain and Spinal Cord Astrocytes of Wild-Type and SOD1 (G93A) Mice.
Cell
, 10
(5)
, Article 1257. 10.3390/cells10051257.
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Schiavo, G;
Greensmith, L;
Hafezparast, M;
Fisher, EM;
(2013)
Cytoplasmic dynein heavy chain: the servant of many masters.
Trends Neurosci
, 36
(11)
pp. 641-651.
10.1016/j.tins.2013.08.001.
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Schottlaender, LV;
Abeti, R;
Jaunmuktane, Z;
Macmillan, C;
Chelban, V;
O'Callaghan, B;
McKinley, J;
... Houlden, H; + view all
(2020)
Bi-allelic JAM2 Variants Lead to Early-Onset Recessive Primary Familial Brain Calcification.
AJHG
, 106
(3)
pp. 412-421.
10.1016/j.ajhg.2020.02.007.
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Smith, AS;
Passey, SL;
Martin, NR;
Player, DJ;
Mudera, V;
Greensmith, L;
Lewis, MP;
(2016)
Creating Interactions between Tissue-Engineered Skeletal Muscle and the Peripheral Nervous System.
Cells Tissues Organs
, 202
(3-4)
pp. 143-158.
10.1159/000443634.
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Soares, D;
Goldrick, I;
Lemon, RN;
Kraskov, A;
Greensmith, L;
Kalmar, B;
(2017)
Expression of Kv3.1b potassium channel is widespread in macaque motor cortex pyramidal cells: A histological comparison between rat and macaque.
Journal of Comparative Neurology
, 525
(9)
pp. 2164-2174.
10.1002/cne.24192.
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Spicer, C;
Lu, C-H;
Catapano, F;
Scoto, M;
Zaharieva, I;
Malaspina, A;
Morgan, JE;
... Zhou, H; + view all
(2021)
The altered expression of neurofilament in mouse models and patients with spinal muscular atrophy.
Annals of Clinical and Translational Neurology
10.1002/acn3.51336.
(In press).
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Stevens, JC;
Chia, R;
Hendriks, WT;
Bros-Facer, V;
van Minnen, J;
Martin, JE;
Jackson, GS;
... Fisher, EMC; + view all
(2010)
Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag - Implications for Research into Amyotrophic Lateral Sclerosis (ALS).
PLOS ONE
, 5
(3)
, Article e9541. 10.1371/journal.pone.0009541.
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Suetterlin, K;
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