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Dr Mark Cooper

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Personal Profile

Name: Mark Cooper Email: jmark.cooper@ucl.ac.uk
Title: Dr Tel: 020 74726604
Department: Clinical Neuroscience Fax: 020 7472 6829
Position: Reader in Neurology Address: Rowland Hill Street, London, London, NW3 2PF
Research Domain: Ageing, Basic Life Sciences, Neuroscience Web Page:  

Profile

Research Description

Parkinson’s Disease (PD):My research focuses on elucidating the cell and molecular mechanisms underlying Parkinson’s disease. Over the past  20 years my research has focussed on the role of mitochondrial dysfunction in PD brains (Schapira et al 1990)  and platelets (Krige et al 1994) and its relationship to other features detected in PD brains including ; mitochondrial DNA defects (Gu et al 1998 ),  nitric oxide (Cleeter et al 1994), oxidative stress (Seaton et al 1997), iron (Hartley et al 1993) and cell death (Hartley et al 1994).  Increasingly with the identification of the familial causes of PD my research has turned to the role of specific mutant genes in PD pathogenesis. Alpha-synuclein has been my main focus with additional interests in ; LRRK2 (Papkovskaia et al 2012), parkin (Gegg et al 2010) and glucocerebrosidase (Gegg et al 2012). While mitochondrial biochemistry is a recurrent theme I also have a broader interest including dopamine metabolism (Tabrizi et al 2000), protein degradation pathways (Alvarez-Erviti et al 2010), alpha-synuclein aggregation and transmission (Alvarez-Erviti et al 2011) and microRNA dysregulation (Alvarez-Erviti et al 2013).

Alpha-synuclein : alpha-synuclein pathology is at the core of PD. I am interested in the normal function of alpha-synuclein. I have a project investigating the regulation of S129 phosphorylation, (Chau et al 2009) and how it may affect its function.I am interested in the cellular handling of alpha-synuclein in particular the pathways of its degradation and how these may be affected in PD. We have identified a deficiency of hsc70 and LAMP2A proteins in PD brains (Alvarez-Erviti et al 2010) which may be responsible for the cellular increase in alpha-synuclein levels in PD.   In addition we are studying what factors influence its; oligomerisation, aggregation and  transmission of synuclein aggregates between cells and the role of exosomes in this process (Alvarez-Erviti et al 2011) . In collaboration with Lydia Alvarez I have a project investigating the role of microRNA dysregulation in PD and how this relates to other pathological findings. We have identified specific microRNAs that can regulate hsc70 and LAMP2A expression which are dramatically elevated in PD brains possibly explaining the observed changes (Alvarez-Erviti et al 2013). We are now investigating the mechanisms underpinning the microRNA dysregulation. I have a collaboration with Lydia Alvarez studying the potential of RVG modified exosome as an in vivo vehicle for the delivery of siRNAs to murine CNS to down-regulate alpha-synuclein.  This is using various cell lines and the human S129D alpha-synuclein transgenic mouse model which shows alpha-synuclein aggregation. This is an exciting new system which has the potential to treat PD by preventing the increased alpha-synuclein aggregation and its transmission between neurons with disease progression.

 


Research Activities

A systematic investigation into the pathogenesis and course of Parkinson's syndrome

Generation of a cell model of a novel SNCA mutation

Models of Neurodegenerative Disease

Neurodegenerative diseases, in particular the investigation of the molecular mechanisms of Parkinson's

Education Description

I coordinate and  lecture on the Genes to neurodegenerative disease  BSc module (BIOC3016),  and MSc module (BIOCM016).
 I supervise MSc library projects (Molecular Medicine MSc) and supervise laboratory projects for BSc, MSc and MRES students on the Biochemistry, Biomedicine  and Neuroscience BSc and Clinical Neuroscience  and Molecular Medicine MSc.

UCL Collaborators

Prof Tarek Yousry; Prof John Hardy; Dr Jose Tomas Bras; Dr Laura Silveira Moriyama; Dr Andrey Abramov; Prof Andrew Lees; Dr Helen Ling; Dr Jan-Willem Taanman; Dr Derralynn Hughes; Prof Rhona Mirsky; Prof Michael Duchen; Prof Kailash Bhatia; Dr Paul Simons; Prof Tony Schapira; Dr Rita Louro Guerreiro; Dr Christos Proukakis; Prof Nicholas Wood; Prof Tamas Revesz; Prof Justin Hsuan

External Collaborators

Dr Dario Alessi; Dr James Martin Staddon; Dr Alexander James Whitworth; Dr Atul Mehta

Publications

    2014

    • McNeill A, Magalhaes J, Shen C, Chau KY, Hughes D, Mehta A, Foltynie T, Cooper JM, Abramov AY, Gegg M, Schapira AH (2014). Ambroxol improves lysosomal biochemistry in glucocerebrosidase mutation-linked Parkinson disease cells.. Brain, , - . doi:10.1093/brain/awu020
    • Cooper JM, Wiklander PB, Nordin JZ, Al-Shawi R, Wood MJ, Vithlani M, Schapira AH, Simons JP, El-Andaloussi S, Alvarez-Erviti L (2014). Systemic exosomal siRNA delivery reduced alpha-synuclein aggregates in brains of transgenic mice.. Mov Disord, , - . doi:10.1002/mds.25978
    • Nalls MA, Saad M, Noyce AJ, Keller MF, Schrag A, Bestwick JP, Traynor BJ, Gibbs JR, Hernandez DG, Cookson MR, Morris HR, Williams N, Gasser T, Heutink P, Wood N, Hardy J, Martinez M, Singleton AB (2014). Genetic comorbidities in Parkinson's disease. Hum.Mol.Genet., 23(3), 831 - 841. doi:10.1093/hmg/ddt465
    • Beilina A, Rudenko IN, Kaganovich A, Civiero L, Chau H, Kalia SK, Kalia LV, Lobbestael E, Chia R, Ndukwe K, Ding J, Nalls MA, Olszewski M, Hauser DN, Kumaran R, Lozano AM, Baekelandt V, Greene LE, Taymans JM, Greggio E, Cookson MR (2014). Unbiased screen for interactors of leucine-rich repeat kinase 2 supports a common pathway for sporadic and familial Parkinson disease. Proc.Natl.Acad.Sci.U.S.A, 111(7), 2626 - 2631. doi:10.1073/pnas.1318306111

    2013

    • Pichler I, Del Greco MF, Gogele M, Lill CM, Bertram L, Do CB, Eriksson N, Foroud T, Myers RH, Nalls M, Keller MF, Benyamin B, Whitfield JB, Pramstaller PP, Hicks AA, Thompson JR, Minelli C (2013). Serum iron levels and the risk of Parkinson disease: a Mendelian randomization study. PLoS.Med., 10(6), e1001462 - . doi:10.1371/journal.pmed.1001462
    • Chau KY, Cooper JM, Schapira AH (2013). Pramipexole reduces phosphorylation of α-synuclein at serine-129.. J Mol Neurosci, 51(2), 573 - 580. doi:10.1007/s12031-013-0030-8
    • Alvarez-Erviti L, Seow Y, Schapira AH, Rodriguez-Oroz MC, Obeso JA, Cooper JM (2013). Influence of microRNA deregulation on chaperone-mediated autophagy and α-synuclein pathology in Parkinson's disease.. Cell Death Dis, 4, e545 - . doi:10.1038/cddis.2013.73
    • Metz G, Coppard N, Cooper JM, Delatycki MB, Dürr A, Di Prospero NA, Giunti P, Lynch DR, Schulz JB, Rummey C, Meier T (2013). Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database.. Brain, 136(Pt 1), 259 - 268. doi:10.1093/brain/aws309
    • Klebe S, Golmard JL, Nalls MA, Saad M, Singleton AB, Bras JM, Hardy J, Simon-Sanchez J, Heutink P, Kuhlenbäumer G, Charfi R, Klein C, Hagenah J, Gasser T, Wurster I, Lesage S, Lorenz D, Deuschl G, Durif F, Pollak P, Damier P, Tison F, Durr A, Amouyel P, Lambert JC, Tzourio C, Maubaret C, Charbonnier-Beaupel F, Tahiri K, Vidailhet M, Martinez M, Brice A, Corvol JC, French Parkinson's Disease Genetics Study Group , International Parkinson's Disease Genomics Consortium (IPDGC) (2013). The Val158Met COMT polymorphism is a modifier of the age at onset in Parkinson's disease with a sexual dimorphism.. J Neurol Neurosurg Psychiatry, 84(6), 666 - 673. doi:10.1136/jnnp-2012-304475
    • Metz G, Coppard N, Meier T, Cooper JM, Delatycki MB, Dürr A, Di Prospero NA, Giunti P, Lynch DR, Schulz JB, Rummey C (2013). Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: Analysis of a 603-patient database. Brain, 136(1), 259 - 268. doi:10.1093/brain/aws309
    • Cleeter MW, Chau KY, Gluck C, Mehta A, Hughes DA, Duchen M, Wood NW, Hardy J, Mark Cooper J, Schapira AH (2013). Glucocerebrosidase inhibition causes mitochondrial dysfunction and free radical damage.. Neurochem Int, 62(1), 1 - 7. doi:10.1016/j.neuint.2012.10.010
    • Holmans P, Moskvina V, Jones L, Sharma M, International Parkinson's Disease Genomics Consortium , Vedernikov A, Buchel F, Saad M, Bras JM, Bettella F, Nicolaou N, Simón-Sánchez J, Mittag F, Gibbs JR, Schulte C, Durr A, Guerreiro R, Hernandez D, Brice A, Stefánsson H, Majamaa K, Gasser T, Heutink P, Wood NW, Martinez M, Singleton AB, Nalls MA, Hardy J, Morris HR, Williams NM (2013). A pathway-based analysis provides additional support for an immune-related genetic susceptibility to Parkinson's disease.. Hum Mol Genet, 22(5), 1039 - 1049. doi:10.1093/hmg/dds492

    2012

    • Keller MF, Saad M, Bras J, Bettella F, Nicolaou N, Simón-Sánchez J, Mittag F, Büchel F, Sharma M, Gibbs JR, Schulte C, Moskvina V, Durr A, Holmans P, Kilarski LL, Guerreiro R, Hernandez DG, Brice A, Ylikotila P, Stefánsson H, Majamaa K, Morris HR, Williams N, Gasser T, Heutink P, Wood NW, Hardy J, Martinez M, Singleton AB, Nalls MA, International Parkinson's Disease Genomics Consortium (IPDGC) , Wellcome Trust Case Control Consortium 2 (WTCCC2) (2012). Using genome-wide complex trait analysis to quantify 'missing heritability' in Parkinson's disease.. Hum Mol Genet, 21(22), 4996 - 5009. doi:10.1093/hmg/dds335
    • Cnop M, Igoillo-Esteve M, Rai M, Begu A, Serroukh Y, Depondt C, Musuaya AE, Marhfour I, Ladrière L, Moles Lopez X, Lefkaditis D, Moore F, Brion JP, Cooper JM, Schapira AH, Clark A, Koeppen AH, Marchetti P, Pandolfo M, Eizirik DL, Féry F (2012). Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes.. Ann Neurol, 72(6), 971 - 982. doi:10.1002/ana.23698
    • Papkovskaia TD, Chau KY, Inesta-Vaquera F, Papkovsky DB, Healy DG, Nishio K, Staddon J, Duchen MR, Hardy J, Schapira AH, Cooper JM (2012). G2019S leucine-rich repeat kinase 2 causes uncoupling protein-mediated mitochondrial depolarization.. Hum Mol Genet, 21(19), 4201 - 4213. doi:10.1093/hmg/dds244
    • Gegg ME, Burke D, Heales SJ, Cooper JM, Hardy J, Wood NW, Schapira AH (2012). Glucocerebrosidase deficiency in substantia nigra of parkinson disease brains. Ann.Neurol., 72(3), 455 - 463. doi:10.1002/ana.23614

    2011

    • Nalls MA, Plagnol V, Hernandez DG, Sharma M, Sheerin UM, Saad M, Simon-Sanchez J, Schulte C, Lesage S, Sveinbjornsdottir S, Arepalli S, Barker R, Ben-Shlomo Y, Berendse HW, Berg D, Bhatia K, de Bie RMA, Biffi A, Bloem B, Bochdanovits Z, Bonin M, Bras JM, Brockmann K, Brooks J, Burn DJ, Charlesworth G, Chen HL, Chinnery PF, Chong S, Clarke CE, Cookson MR, Cooper JM, Corvol JC, Counsell C, Damier P, Dartigues JF, Deloukas P, Deuschl G, Dexter DT, van Dijk KD, Dillman A, Durif F, Durr A, Edkins S, Evans JR, Foltynie T, Gao JJ, Gardner M, Gibbs JR, Goate A, Gray E, Guerreiro R, Gustafsson O, Harris C, van Hilten JJ, Hofman A, Hollenbeck A, Holton J, Hu M, Huang XM, Huber H, Hudson G, Hunt SE, Huttenlocher J, Illig T, Jonsson PV, Lambert JC, Langford C, Lees A, Lichtner P, Limousin P, Lopez G, Lorenz D, McNeill A, Moorby C, Moore M, Morris HR, Morrison KE, Mudanohwo E, O'Sullivan SS, Pearson J, Perlmutter JS, Petursson H, Pollak P, Post B, Potter S, Ravina B, Revesz T, Riess O, Rivadeneira F, Rizzu P, Ryten M, Sawcer S, Schapira A, Scheffer H, Shaw K, Shoulson I, Sidransky E, Smith C, Spencer CCA, Stefansson H, Stockton JD, Strange A, Talbot K, Tanner CM, Tashakkori-Ghanbaria A, Tison F, Trabzuni D, Traynor BJ, Uitterlinden AG, Velseboer D, Vidailhet M, Walker R, van de Warrenburg B, Wickremaratchi M, Williams N, Williams-Gray CH, Winder-Rhodes S, Stefansson K, Martinez M, Hardy J, Heutink P, Brice A, Gasser T, Singleton AB, Wood NW, Int Parkinson Dis Genomics Consort , Wellcome Trust Case-Control Consor (2011). Imputation of sequence variants for identification of genetic risks for Parkinson's disease: a meta-analysis of genome-wide association studies. LANCET, 377(9766), 641 - 649. doi:10.1016/S0140-6736(10)62345-8
    • Plagnol V, Nalls MA, Bras JM, Hernandez DG, Sharma M, Sheerin UM, Saad M, Simon-Sanchez J, Schulte C, Lesage S, Sveinbjornsdottir S, Amouyel P, Arepalli S, Band G, Barker RA, Bellinguez C, Ben-Shlomo Y, Berendse HW, Berg D, Bhatia K, de Bie RMA, Biffi A, Bloem B, Bochdanovits Z, Bonin M, Brockmann K, Brooks J, Burn DJ, Charlesworth G, Chen HL, Chinnery PF, Chong S, Clarke CE, Cookson MR, Cooper JM, Corvol JC, Counsell C, Damier P, Dartigues JF, Deloukas P, Deuschl G, Dexter DT, van Dijk KD, Dillman A, Durif F, Durr A, Edkins S, Evans JR, Foltynie T, Freeman C, Gao JJ, Gardner M, Gibbs JR, Goate A, Gray E, Guerreiro R, Gustafsson O, Harris C, Hellenthal G, van Hilten JJ, Hofman A, Hollenbeck A, Holton J, Hu M, Huang XM, Huber H, Hudson G, Hunt SE, Huttenlocher J, Illig T, Jonsson PV, Langford C, Lees A, Lichtner P, Limousin P, Lopez G, Lorenz D, McNeill A, Moorby C, Moore M, Morris H, Morrison KE, Mudanohwo E, O'Sullivan SS, Pearson J, Pearson R, Perlmutter JS, Petursson H, Pirinen M, Pollak P, Post B, Potter S, Ravina B, Revesz T, Riess O, Rivadeneira F, Rizzu P, Ryten M, Sawcer S, Schapira A, Scheffer H, Shaw K, Shoulson I, Sidransky E, de Silva R, Smith C, Spencer CCA, Stefansson H, Steinberg S, Stockton JD, Strange A, Su Z, Talbot K, Tanner CM, Tashakkori-Ghanbaria A, Tison F, Trabzuni D, Traynor BJ, Uitterlinden AG, Vandrovcova J, Velseboer D, Vidailhet M, Vukcevic D, Walker R, van de Warrenburg B, Weale ME, Wickremaratchi M, Williams N, Williams-Gray CH, Winder-Rhodes S, Stefansson K, Martinez M, Donnelly P, Singleton AB, Hardy J, Heutink P, Brice A, Gasser T, Wood NW, WTCCC2 (2011). A Two-Stage Meta-Analysis Identifies Several New Loci for Parkinson's Disease. PLOS GENET, 7(6), - . doi:10.1371/journal.pgen.1002142
    • Alvarez-Erviti L, Seow Y, Schapira AH, Gardiner C, Sargent IL, Wood MJ, Cooper JM (2011). Lysosomal dysfunction increases exosome-mediated alpha-synuclein release and transmission.. Neurobiol Dis, 42(3), 360 - 367. doi:10.1016/j.nbd.2011.01.029
    • Alvarez-Erviti L, Couch Y, Richardson J, Cooper JM, Wood MJ (2011). Alpha-synuclein release by neurons activates the inflammatory response in a microglial cell line.. Neurosci Res, 69(4), 337 - 342. doi:10.1016/j.neures.2010.12.020

    2010

    • Rajagopalan B, Francis JM, Cooke F, Korlipara LVP, Blamire AM, Schapira AHV, Madan J, Neubauer S, Cooper JM (2010). Analysis of the Factors Influencing the Cardiac Phenotype in Friedreich's Ataxia. MOVEMENT DISORD, 25(7), 846 - 852. doi:10.1002/mds.22864
    • Chau KY, Cooper JM, Schapira AH (2010). Rasagiline protects against alpha-synuclein induced sensitivity to oxidative stress in dopaminergic cells.. Neurochem Int, 57(5), 525 - 529. doi:10.1016/j.neuint.2010.06.017
    • Gegg ME, Cooper JM, Chau KY, Rojo M, Schapira AH, Taanman JW (2010). Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy.. Hum Mol Genet, 19(24), 4861 - 4870. doi:10.1093/hmg/ddq419
    • Alvarez-Erviti L, Rodriguez-Oroz MC, Cooper JM, Caballero C, Ferrer I, Obeso JA, Schapira AHV (2010). Chaperone-Mediated Autophagy Markers in Parkinson Disease Brains. ARCH NEUROL-CHICAGO, 67(12), 1464 - 1472. doi:10.1001/archneuro1.2010.198

    2009

    • Cano SJ, Riazi A, Schapira AH, Cooper JM, Hobart JC (2009). Friedreich's ataxia impact scale: a new measure striving to provide the flexibility required by today's studies. Mov Disord., 24(7), 984 - 992.
    • Chau KY, Ching HL, Schapira AH, Cooper JM (2009). Relationship between alpha synuclein phosphorylation, proteasomal inhibition and cell death: relevance to Parkinson's disease pathogenesis. J.Neurochem., 110(3), 1005 - 1013.
    • Chau KY, Korlipara LV, Cooper JM, Schapira AH (2009). Protection against paraquat and A53T alpha-synuclein toxicity by cabergoline is partially mediated by dopamine receptors. J.Neurol.Sci., 278(1-2), 44 - 53.
    • Gegg ME, Cooper JM, Schapira AH, Taanman JW (2009). Silencing of PINK1 expression affects mitochondrial DNA and oxidative phosphorylation in dopaminergic cells. PLoS.One., 4(3), e4756 - .

    2008

    • Cooper JM, Korlipara LVP, Hart PE, Bradley JL, Schapira AHV (2008). Coenzyme Q10 and vitamin E therapy in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy.. European Journal of Neurology, 15(12), 1371 - 1379. doi:10.1111/j.1468-1331.2008.02318.x
    • DiFrancesco JC, Cooper JM, Lam A, Hart PE, Tremolizzo L, Ferrarese C, Schapira AH (2008). MELAS mitochondrial DNA mutation A3243G reduces glutamate transport in cybrids cell lines.. Experimental Neurology, 212(1), 152 - 156. doi:10.1016/j.expneurol.2008.03.015

    2007

    • Turner C, Cooper JM, Schapira AH (2007). Clinical correlates of mitochondrial function in Huntington's disease muscle.. Movement Disorders, 22(12), 1715 - 1721. doi:10.1002/mds.21540
    • Cooper JM, Schapira AH (2007). Friedreich's ataxia: Coenzyme Q(10) and vitamin E therapy.. Mitochondrion, 7(Suppl.1), S127 - S135. doi:10.1016/j.mito.2007.04.001

    2006

    • Al-Mahdawi S, Pinto RM, Varshney D, Lawrence L, Lowrie MB, Hughes S, Webster Z, Blake J, Cooper JM, King R, Pook MA (2006). GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.. Genomics, 88(5), 580 - 590. doi:10.1016/j.ygeno.2006.06.015
    • Cano SJ, Hobart JC, Hart PE, Kolipara LVP, Schapira AHV, Cooper JM (2006). International co-operative ataxia rating scale (ICARS): Suitable for clinical practice and treatment trials in Friedreich's ataxia?.
    • Cano SJ, Riazi A, Cooper JM, Schapira AHV, Hobart JC (2006). The Friedreich's Ataxia impact scale (FAIS) meets the needs of today's clinical studies.
    • Schapira AH, Cleeter MW, Muddle JR, Workman JM, Cooper JM, King RH (2006). Proteasomal inhibition causes loss of nigral tyrosine hydroxylase neurons.. Annals of Neurology, 60(2), 253 - 255. doi:10.1002/ana.20934
    • Iravani MM, Haddon CO, Cooper JM, Jenner P, Schapira AH (2006). Pramipexole protects against MPTP toxicity in non-human primates.. Journal of Neurochemistry, 96(5), 1315 - 1321.
    • Riazi A, Cano SJ, Cooper JM, Bradley JL, Schapira AH, Hobart JC (2006). Coordinating outcomes measurement in ataxia research: Do some widely used generic rating scales tick the boxes?. Movement Disorders, 21(9), 1396 - 1403.

    2005

    • Proukakis C, Cooper JM, Taanman JW, Warner TT (2005). Cellular studies of spastin reveal no gain of function, and suggest translation from the 2nd ATG. EUR J NEUROL, 12, 323 - 323.
    • Cano SJ, Hobart JC, Hart PE, Korlipara LVP, Schapira AH, Cooper JM (2005). International Cooperative Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich's ataxia?. Movement Disorders, 20(12), 1585 - 1591.
    • Gu M, Iravani MM, Cooper JM, King D, Jenner P, Schapira AHV (2005). Pramipexole protects against apoptotic cell death by non-dopaminergic mechanisms (vol 91, pg 1075, 2004). J NEUROCHEM, 92(1), 215 - 215. doi:10.1111/j.1471-4159.2004.02966.x
    • Hart PE, Lodi R, Rajagopalan B, Bradley JL, Crilley JG, Turner C, Blamire AM, Manners D, Styles P, Schapira AH, Cooper JM (2005). Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up.. Archives of Neurology, 62(4), 621 - 626.
    • Salinas S, Carazo-Salas RE, Proukakis C, Cooper JM, Weston AE, Schiavo G, Warner TT (2005). Human spastin has multiple microtubule-related functions.. Journal of Neurochemistry, 95(5), 1411 - 1420. doi:10.1111/j.1471-4159.2005.03472.x
    • Lo S, Tolner B, Taanman JW, Cooper JM, Gu M, Hartley JA, Schapira AH, Hochhauser D (2005). Assessment of the significance of mitochondrial DNA damage by chemotherapeutic agents.. International Journal of Oncology, 27(2), 337 - 344.
    • Misbahuddin A, Placzek MR, Taanman JW, Gschmeissner S, Schiavo G, Cooper JM, Warner TT (2005). Mutant torsinA, which causes early-onset primary torsion dystonia, is redistributed to membranous structures enriched in vesicular monoamine transporter in cultured human SH-SY5Y cells.. Movement Disorders, 20(4), 432 - 440. doi:10.1002/mds.20351

    2004

    • Orth M, Tabrizi SJ, Tomlinson C, Messmer K, Korlipara LVP, Schapira AHV, Cooper JM (2004). G209A mutant alpha synuclein expression specifically enhances dopamine induced oxidative damage. Neurochemistry International, 45(5), 669 - 676. doi:10.1016/j.neuint.2004.03.029
    • Korlipara LVP, Cooper JM, Schapira AHV (2004). Oxidative stress in a neuronal model of alpha synuclein overexpression.
    • Proukakis C, Taanman JW, Cooper JM, Warner TT (2004). Cellular studies of spastin, the protein commonly mutated in autosomal dominant hereditary spastic paraplegia (SPG4).
    • Bradley JL, Homayoun S, Hart PE, Schapira AH, Cooper JM (2004). Role of oxidative damage in Friedreich's ataxia.. Neurochemical Research, 29(3), 561 - 567. doi:10.1023/B:NERE.0000014826.00881.c3
    • Korlipara LV, Cooper JM, Schapira AH (2004). Differences in toxicity of the catechol-O-methyl transferase inhibitors, tolcapone and entacapone to cultured human neuroblastoma cells.. Neuropharmacology, 46(4), 562 - 569.
    • Gu M, Iravani MM, Cooper JM, King D, Jenner P, Schapira AH (2004). Pramipexole protects against apoptotic cell death by non-dopaminergic mechanisms.. Journal of Neurochemistry, 91(5), 1075 - 1081.
    • Rafique R, Schapira AHV, Cooper JM (2004). Mitochondrial respiratory chain dysfunction in ageing; Influence of vitamin E deficiency. Free Radical Research, 38(2), 157 - 165.

    2003

    • Orth M, Tabrizi SJ, Schapira AHV, Cooper JM (2003). alpha-Synuclein expression in HEK293 cells enhances the mitochondrial sensitivity to rotenone. Neuroscience Letters, 351(1), 29 - 32.
    • Lodi R, Rajagopalan B, Schapira AH, Cooper JM (2003). Cardiac bioenergetics in Friedreich's ataxia. Annals of Neurology, 54(4), 552 - 553.
    • Cooper JM, Schapira AHV (2003). Friedreich's Ataxia: Disease mechanisms, antioxidant and coenzyme Q_{10} therapy. Biofactors, 18(1-4), 163 - 171.
    • Orth M, Cooper JM, Bates GP, Schapira AH (2003). Inclusion formation in Huntington's disease R6/2 mouse muscle cultures. Journal of Neurochemistry, 87(1), 1 - 6.
    • Cooper JM (2003). Mitochondrial dysfunction in neurodegenerative disease. In Holt IJ (Ed.), Genetics of Mitochondrial Diseases (pp. 229 - 246). : OU Press.
    • Misbahuddin A, Placzek MR, Taanman JW, Cooper JM, Warner TT (2003). Study of mutant and wild type torsin A in human SH-SY5Y cell lines.
    • Schapira AHV, Gu M, Cooper JM (2003). Dopamine agonist mediated neuroprotection: Mechanisms of action.

    2002

    • Lodi R, Rajagopalan B, Bradley JL, Taylor DJ, Crilley JG, Hart PE, Blamire AM, Manners D, Styles P, Schapira AHV, Cooper JM (2002). Mitochondrial dysfunction in Friedreich's ataxia: From pathogenesis to treatment perspectives. Free Radical Research, 36(4), 461 - 466.
    • Lodi R, Rajagopalan B, Crilley JG, Cooper JM, Styles P, Schapira AHV (2002). Cardiac hypertrophy in Friedreich's ataxia - Reply to letter to the editor. Cardiovascular Research, 54(3), 695 - 696.
    • Bradley JL, Schapira AH, Cooper JM (2002). Therapies for Friedreich's ataxia. EuroAtaxia Newsletter, 22, 8 - 11.
    • Cooper JM, Bradley JL (2002). Friedreich's ataxia. In Schapira AH (Ed.), Mitochondrial Function and Dysfunction, International Review of Neurobiology (pp. 147 - 173). : Academic Press.

    2001

    • Lodi R, Rajagopalan B, Blamire AM, Cooper JM, Davies CH, Bradley JL, Styles P, Schapira AHV (2001). Cardiac energetics are abnormal in Friedreich ataxia patients in the absence of cardiac dysfunction and hypertrophy: An in vivo P-31 magnetic resonance spectroscopy study. Cardiovascular Research, 52(1), 111 - 119. doi:10.1016/S0008-6363(01)00357-1
    • Pook M, Al-Mahdawi S, Carroll C, Cossee M, Puccio H, Lawrence L, Clark P, Lowrie M, Bradley J, Cooper JM, Koenig M, Chamberlain S (2001). Rescue of the frataxin knockout mouse embryonic lethal phenotype by human YAC and BAC transgenesis.. AM J HUM GENET, 69(4), 579 - 579.
    • Kingsbury AE, Cooper M, Schapira AHV, Foster OJF (2001). Metabolic enzyme expression in dopaminergic neurons in Parkinson's disease: An in situ hybridization study. Annals of Neurology, 50(2), 142 - 149.
    • Pook MA, Al Mahdawi S, Carroll CJ, Cossee M, Puccio H, Lawrence L, Clark P, Lowrie MB, Bradley JL, Cooper JM, Koenig M, Chamberlain S (2001). Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis. Neurogenetics, 3(4), 185 - 193.
    • Lodi R, Hart PE, Rajagopalan B, Taylor DJ, Crilley JG, Bradley JL, Blamire AM, Manners D, Styles P, Schapira AHV, Cooper JM (2001). Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia. Annals of Neurology, 49(5), 590 - 596. doi:10.1002/ana.1001
    • Rafique R, Schapira AHV, Cooper JM (2001). Sensitivity of respiratory chain activities to lipid peroxidation: effect of vitamin E deficiency. Biochemical Journal, 357(3), 887 - 892. doi:10.1042/0264-6021:3570887
    • Cleeter MWJ, Cooper JM, Schapira AHV (2001). Nitric oxide enhances MPP+ inhibition of complex I. FEBS LETT, 504(1-2), 50 - 52.

    2000

    • Gu M, Cooper JM, Butler P, Walker AP, Mistry PK, Dooley JS, Schapira AH (2000). Oxidative-phosphorylation defects in liver of patients with Wilson's disease. The Lancet, 356(9228), 469 - 474.
    • Bradley JL, Blake JC, Chamberlain S, Thomas PK, Cooper JM, Schapira AHV (2000). Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia. Human Molecular Genetics, 9(2), 275 - 282.
    • Tabrizi SJ, Orth M, Wilkinson JM, Taanman JW, Warner TT, Cooper JM, Schapira AHV (2000). Expression of mutant alpha-synuclein causes increased susceptibility to dopamine toxicity. Human Molecular Genetics, 9(18), 2683 - 2689.
    • Gu M, Cooper JM, Butler P, Walker AP, Mistry PK, Dooley JS, Schapira AHV (2000). Oxidative phosphorylation defects in Wilson's disease liver. Hepatology, 32(4 Pt 2), 487A - .
    • Tabrizi SJ, Workman J, Hart PE, Mangiarini L, Mahal A, Bates G, Cooper JM, Schapira AHV (2000). Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse. Annals of Neurology, 47(1), 80 - 86.
    • Attimonelli M, Altamura N, Benne R, Brennicke A, Cooper JM, DElia D, deMontalvo A, dePinto B, DeRobertis M, Golik P, Knoop V, Lanave C, Lazowska J, Licciulli F, Malladi BS, Memeo F, Monnerot M, Pasimeni R, Pilbout S, Schapira AHV, Sloof P, Saccone C (2000). MitBASE: a comprehensive and integrated mitochondrial DNA database. The present status. Nucleic Acids Research, 28(1), 148 - 152.
    • Luo ZJ, King RHM, Cooper JM, Thomas PK (2000). Demonstration of neurotropic activity throughout the length of the transected rat sciatic nerve. BRAIN PATHOLOGY, 10(4), 553 - 553.
    • Rahman S, Lake BD, Taanman JW, Hanna MG, Cooper JM, Shapira AHV, Leonard JV (2000). Cytochrome C oxidase immunohistochemistry: Clues for genetic mechanisms. Brain, 123(3), 591 - 600.

    1999

    • Attimonelli M, Cooper JM, Delia D, de Montalvo A, De Robertis M, Lehvaslaiho H, Malladi SB, Memeo F, Stevens K, Schapira AHV, Saccone C (1999). Update of the human MitBASE database. Nucleic Acids Research, 27, 143 - 146.
    • Attimonelli M, Altamura N, Benne R, Boyen C, Brennicke A, Carone A, Cooper JM, D'Elia D, de Montalvo A, de Pinto B, De Robertis M, Golik P, Grienenberger JM, Knoop V, Lanave C, Lazowska J, Lemagnen A, Malladi BS, Memeo F, Monnerot M, Pilbout S, Schapira AH, Sloof P, Slonimski P, Saccone C, et AL (1999). MitBASE: a comprehensive and integrated mitochondrial DNA database.. Nucleic Acids Research, 27(1), 128 - 133.
    • Lodi R, Cooper JM, Bradley JL, Manners D, Styles P, Taylor DJ, Schapira AHV (1999). Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia. Proceedings of the National Academy of Sciences of the United States of America, 96(20), 11492 - 11495.
    • Cock HR, Cooper JM, Schapira AHV (1999). Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy. Journal of the Neurological Sciences, 165(1), 10 - 17.
    • Tabrizi SJ, Cleeter MW, Xuereb J, Taanman JW, Cooper JM, Schapira AH (1999). Biochemical abnormalities and excitotoxicity in Huntington's disease brain.. Annals of Neurology, 45(1), 25 - 32.
    • Blake JC, Taanman JW, Morris AM, Gray RG, Cooper JM, McKiernan PJ, Leonard JV, Schapira AH (1999). Mitochondrial DNA depletion syndrome is expressed in amniotic fluid cell cultures.. American Journal of Pathology, 155(1), 67 - 70.
    • Rahman S, Taanman JW, Cooper JM, Nelson I, Hargreaves I, Meunier B, Hanna MG, Garcia JJ, Capaldi RA, Lake BD, Leonard JV, Schapira AHV (1999). A missense mutation of cytochrome oxidase subunit II causes defective assembly and myopathy. The American Journal of Human Genetics, 65(4), 1030 - 1039.

    1998

    • Tabrizi SJ, Cooper JM, Schapira AHV (1998). Mitochondrial DNA in focal dystonia: a cybrid analysis. Annals of Neurology, 44(2), 258 - 261.
    • Morris AAM, Taanman JW, Blake J, Copper JM, Lake BD, Malone M, Love S, Clayton P, Leonard JV, Schapira AHV (1998). Liver failure associated with mitochondrial DNA depletion. Journal of Hepatology, 28, 556 - 563.
    • Gu M, Cooper JM, Taanman JW, Schapira AHV (1998). Mitochondrial DNA transmission of the mitochondrial defect in Parkinson's disease. Annals of Neurology, 44(2), 177 - 186.
    • Gu M, Owen AD, Toffa SEK, Cooper JM, Dexter DT, Jenner P, Marsden CD, Schapira AHV (1998). Mitochondrial function, GSH and iron in neurodegeneration and Lewy body diseases. Journal of the Neurological Sciences, 158, 24 - 29.
    • Cock H, Tabrizi SJ, Cooper JM, Schapira AHV (1998). The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathy. Annals of Neurology, 44(2), 187 - 193.
    • Attimonelli M, Calo D, Cooper JM, de Montalvo A, Licciulli F, Sasanelli D, Stevens K, Malladi BS, Saccone C, Schapira AHV (1998). The mitBASE human dataset structure. Nucleic Acids Research, 26(1), 116 - 119.
    • Hanna MG, Nelson IP, Rahman S, Lane RJM, Land J, Heales S, Cooper MJ, Schapira AHV, Morgan-Hughes JA, Wood NW (1998). Cytochrome c oxidase deficiency associated with the first stop-codon point mutation in human mtDNA. The American Journal of Human Genetics, 63(1), 29 - 36.
    • Turner LF, Kaddoura S, Harrington D, Cooper JM, Poole-Wilson PA, Schapira AH (1998). Mitochondrial DNA in idiopathic cardiomyopathy.. European Heart Journal, 19(11), 1725 - 1729.
    • Schapira AHV, Gu M, Taanman JW, Tabrizi SJ, Seaton T, Cleeter M, Cooper JM (1998). Mitochondria in the etiology and pathogenesis of Parkinson's disease. Annals of Neurology, 44 (S1, S89 - S98.
    • Seaton TA, Cooper JM, Schapira AHV (1998). Cyclosporin inhibition of apoptosis induced by mitochondrial complex I toxins. BRAIN RES, 809(1), 12 - 17.

    1997

    • Cooper JM, Schapira AHV (1997). Mitochondrial dysfunction in neurodegeneration. Journal of Bioenergetics and Biomembranes, 29(2), 175 - 183.
    • Schapira AHV, Warner T, Gash MT, Cleeter MWJ, Marinho CFM, Cooper JM (1997). Complex I function in familial and sporadic dystonia. Annals of Neurology, 41, 556 - 559.
    • Seaton TA, Cooper JM, Schapira AH (1997). Free radical scavengers protect dopaminergic cell lines from apoptosis induced by complex I inhibitors.. Brain Research, 777(1-2), 110 - 118.
    • Taanman JW, Bodnar AG, Cooper JM, Morris AAM, Clayton PT, Leonard JV, Schapira AHV (1997). Molecular mechanisms in mitochondrial DNA depletion syndrome. Human Molecular Genetics, 6(6), 935 - 942.
    • Gu M, Gash MT, Cooper JM, Wenning GK, Daniel SE, Quinn NP, Marsden CD, Schapira AHV (1997). Mitochondrial respiratory chain function in multiple system atrophy. Movement Disorders, 12(3), 418 - 422.

    1996

    • Cooper JM, Schapira AH (1996). Biochemical investigation of mitochondrial respiratory chain abnormalities. In Lane R (Ed.), Hand Bood of Muscle Disease (pp. 519 - ). : Marcel Dekker.
    • Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AH (1996). Mitochondrial defect in Huntington's disease caudate nucleus.. Annals of Neurology, 39(3), 385 - 389.
    • Shergill JK, Cammack R, Cooper CE, Cooper JM, Mann VM, Schapira AH (1996). Detection of nitrosyl complexes in human substantia nigra, in relation to Parkinson's disease.. Biochemical and Biophysical Research Communications, 228(2), 298 - 305.

    1995

    • Morgan-Hughes JA, Sweeney MG, Cooper JM, Hammans SR, Brockington M, Schapira AH, Harding AE, Clark JB (1995). Mitochondrial DNA (mtDNA) diseases: correlation of genotype to phenotype.. Biochimica et Biophysica Acta (BBA) - Bioenergetics, 1271(1), 135 - 140.
    • Cock HR, Cooper JM, Schapira AH (1995). The 14484 ND6 mtDNA mutation in Leber hereditary optic neuropathy does not affect fibroblast complex I activity.. The American Journal of Human Genetics, 57(6), 1501 - 1502.
    • Bodnar AG, Cooper JM, Leonard JV, Schapira AH (1995). Respiratory-deficient human fibroblasts exhibiting defective mitochondrial DNA replication.. Biochemical Journal, 305(3), 817 - 822.
    • Hanna MG, Nelson I, Sweeney MG, Cooper JM, Watkins PJ, Morgan-Hughes JA, Harding AE (1995). Congenital encephalomyopathy and adult-onset myopathy and diabetes mellitus: different phenotypic associations of a new heteroplasmic mtDNA tRNA glutamic acid mutation.. Am J Hum Genet, 56(5), 1026 - 1033.
    • Cooper JM, Daniel SE, Marsden CD, Schapira AH (1995). L-dihydroxyphenylalanine and complex I deficiency in Parkinson's disease brain.. Mov Disord, 10(3), 295 - 297.
    • Smith PR, Cooper JM, Govan GG, Riordan-Eva P, Harding AE, Schapira AH (1995). Antibodies to human optic nerve in Leber's hereditary optic neuropathy.. Journal of the Neurological Sciences, 130(2), 134 - 138.

    1994

    • Schapira AH, Cooper JM (1994). Inborn and induced defects of the mitochondrial respiratory chain. Biochem.Soc.Trans., 22(4), 996 - 1001.
    • Schapira AH, Cooper JM (1994). Biochemical and molecular features of deficiencies of complex I,II and III. In Schapira AHD, S (Ed.), (pp. 75 - 90). : Butterworth-Heinemann.
    • Cooper JM, Clarke JB (1994). The structural organisaton of the mitochondrial respiratory chain.. In (Ed.), Mitochondrial Disorders in Neurology (pp. 1 - 30). : Butterworth-Heinemann.
    • Sweeney MG, Hammans SR, Duchen LW, Cooper JM, Schapira AH, Kennedy CR, Jacobs JM, Youl BD, Morgan-Hughes JA, Harding AE (1994). Mitochondrial DNA mutation underlying Leigh's syndrome: clinical, pathological, biochemical, and genetic studies of a patient presenting with progressive myoclonic epilepsy. J.Neurol.Sci., 121(1), 57 - 65.
    • Dexter DT, Sian J, Rose S, Hindmarsh JG, Mann VM, Cooper JM, Wells FR, Daniel SE, Lees AJ, Schapira AH (1994). Indices of oxidative stress and mitochondrial function in individuals with incidental Lewy body disease. Annals of Neurology, 35(1), 38 - 44.
    • Cooper JM, Clark JB (1994). Abnormalities of mitochondrial respiratory chain complexes I-III in humans.. In Darley-Usmar V, Schapira AH (Ed.), (pp. - ). : Portland Press.
    • Smith PR, Cooper JM, Govan GG, Harding AE, Schapira AH (1994). Platelet mitochondrial function in Leber's hereditary optic neuropathy. J.Neurol.Sci., 122(1), 80 - 83.
    • Mann VM, Cooper JM, Daniel SE, Srai K, Jenner P, Marsden CD, Schapira AH (1994). Complex I, iron, and ferritin in Parkinson\'s disease substantia nigra. Ann.Neurol., 36(6), 876 - 881.
    • Hartley A, Stone JM, Heron C, Cooper JM, Schapira AH (1994). Complex I inhibitors induce dose-dependent apoptosis in PC12 cells: relevance to Parkinson's disease. J.Neurochem., 63(5), 1987 - 1990.
    • Taylor DJ, Krige D, Barnes PR, Kemp GJ, Carroll MT, Mann VM, Cooper JM, Marsden CD, Schapira AH (1994). A 31P magnetic resonance spectroscopy study of mitochondrial function in skeletal muscle of patients with Parkinson's disease. J.Neurol.Sci., 125(1), 77 - 81.
    • Cleeter MW, Cooper JM, Darley-Usmar VM, Moncada S, Schapira AH (1994). Reversible inhibition of cytochrome c oxidase, the terminal enzyme of the mitochondrial respiratory chain, by nitric oxide. Implications for neurodegenerative diseases. FEBS Lett., 345(1), 50 - 54.

    1993

    • Smith PR, Cooper JM, Govan GG, Harding AE, Schapira AH (1993). Smoking and mitochondrial function: a model for environmental toxins. Q.J.Med., 86(10), 657 - 660.
    • Harley A, Cooper JM, Schapira AH (1993). Iron induced oxidative stress and mitochondrial dysfunction: relevance to Parkinson's disease. Brain Res., 627(2), 349 - 353.
    • Morten KJ, Cooper JM, Brown GK, Lake BD, Pike E, Poulton J (1993). A new point mutation associated with mitochondrial encephalomyopathy. HUM.MOL.GENET., 2(12), 2081 - 2087.
    • Thomas PK, Cooper JM, King RH, Workman JM, Schapira AH, Goss-Sampson MA, Muller DP (1993). Myopathy in vitamin E deficient rats: muscle fibre necrosis associated with disturbances of mitochondrial function. J.Anat., 183 ( Pt 3), 451 - 461.
    • Cooper JM, Wischik C, Schapira AH (1993). Mitochondrial function in Alzheimer's disease. Lancet, 341(8850), 969 - 970.
    • Bodnar AG, Cooper JM, Holt IJ, Leonard JV, Schapira AH (1993). Nuclear complementation restores mtDNA levels in cultured cells from a patient with mtDNA depletion. Am.J.Hum.Genet., 53(3), 663 - 669.
    • Schapira AH, Hartley A, Cleeter MW, Cooper JM (1993). Free radicals and mitochondrial dysfunction in Parkinson's disease. Biochem.Soc.Trans., 21(2), 367 - 370.

    1992

    • Cooper JM, Mann VM, Schapira AH (1992). Analyses of mitochondrial respiratory chain function and mitochondrial DNA deletion in human skeletal muscle: effect of ageing. J.Neurol.Sci., 113(1), 91 - 98.
    • Mann VM, Cooper JM, Schapira AH (1992). Quantitation of a mitochondrial DNA deletion in Parkinson's disease. FEBS Lett., 299(3), 218 - 222.
    • KRIGE D, CARROLL MT, COOPER JM, MARSDEN CD, SCHAPIRA AHV (1992). PLATELET MITOCHONDRIAL-FUNCTION IN PARKINSONS-DISEASE. ANN NEUROL, 32(6), 782 - 788.
    • Schapira AH, Cooper JM (1992). Mitochondrial function in neurodegeneration and ageing. Mutat.Res., 275(3-6), 133 - 143.
    • Cooper JM, Hayes DJ, Challiss RA, Morgan-Hughes JA, Clark JB (1992). Treatment of experimental NADH ubiquinone reductase deficiency with menadione. Brain, 115 ( Pt 4), 991 - 1000.
    • Cooper JM, Mann VM, Krige D, Schapira AH (1992). Human mitochondrial complex I dysfunction. Biochim.Biophys.Acta, 1101(2), 198 - 203.
    • Haines AM, Cooper JM, Morgan-Hughes JA, Clark JB, Schapira AH (1992). One-step immunoaffinity purification of complex I subunits from beef heart mitochondria. Protein Expr.Purif., 3(3), 223 - 227.
    • Mann VM, Cooper JM, Krige D, Daniel SE, Schapira AH, Marsden CD (1992). Brain, skeletal muscle and platelet homogenate mitochondrial function in Parkinson's disease. Brain, 115 ( Pt 2), 333 - 342.
    • Cleeter MW, Cooper JM, Schapira AH (1992). Irreversible inhibition of mitochondrial complex I by 1-methyl-4-phenylpyridinium: evidence for free radical involvement. J.Neurochem., 58(2), 786 - 789.
    • Hammans SR, Sweeney MG, Holt IJ, Cooper JM, Toscano A, Clark JB, Morgan-Hughes JA, Harding AE (1992). Evidence for intramitochondrial complementation between deleted and normal mitochondrial DNA in some patients with mitochondrial myopathy. J.Neurol.Sci., 107(1), 87 - 92.
    • SCHAPIRA AHV, MANN VM, COOPER JM, KRIGE D, JENNER PJ, MARSDEN CD (1992). MITOCHONDRIAL-FUNCTION IN PARKINSONS-DISEASE.

    1991

    • Morgan-Hughes JA, Cooper JM, Schapira AH, Sweeny M, Holt IJ, Harding AE, Clark JB (1991). The molecular pathology of human respiratory chain defects. Rev.Neurol.(Paris), 147(6-7), 450 - 454.

    1990

    • Schapira AH, Mann VM, Cooper JM, Dexter D, Daniel SE, Jenner P, Clark JB, Marsden CD (1990). Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease. J.Neurochem., 55(6), 2142 - 2145.
    • Harding AE, Holt IJ, Cooper JM, Schapira AH, Sweeney M, Clark JB, Morgan-Hughes JA (1990). Mitochondrial myopathies: genetic defects. Biochem.Soc.Trans., 18(4), 519 - 522.
    • Mann VM, Cooper JM, Javoy-Agid F, Agid Y, Jenner P, Schapira AH (1990). Mitochondrial function and parental sex effect in Huntington's disease. Lancet, 336(8717), 749 - .
    • Schapira AH, Cooper JM, Dexter D, Clark JB, Jenner P, Marsden CD (1990). Mitochondrial complex I deficiency in Parkinson's disease. J.Neurochem., 54(3), 823 - 827.
    • Schapira AH, Cooper JM, Manneschi L, Vital C, Morgan-Hughes JA, Clark JB (1990). A mitochondrial encephalomyopathy with specific deficiencies of two respiratory chain polypeptides and a circulating autoantibody to a mitochondrial matrix protein. Brain, 113 ( Pt 2), 419 - 432.
    • Schapira AH, Cooper JM, Morgan-Hughes JA, Landon DN, Clark JB (1990). Mitochondrial myopathy with a defect of mitochondrial-protein transport. N.Engl.J.Med., 323(1), 37 - 42.
    • Morgan-Hughes JA, Schapira AH, Cooper JM, Holt IJ, Harding AE, Clark JB (1990). The molecular pathology of respiratory-chain dysfunction in human mitochondrial myopathies. Biochim.Biophys.Acta, 1018(2-3), 217 - 222.
    • Cooper JM, Schapira AH, Holt IJ, Toscano A, Harding AE, Morgan-Hughes JA, Clark JB (1990). Biochemical and molecular aspects of human mitochondrial respiratory chain disorders. Biochem.Soc.Trans., 18(4), 517 - 519.
    • Morgan-Hughes JA, Cooper JM, Holt IJ, Harding AE, Schapira AH, Clark JB (1990). Mitochondrial myopathies: clinical defects. Biochem.Soc.Trans., 18(4), 523 - 526.

    1989

    • Schapira AH, Cooper JM, Dexter D, Jenner P, Clark JB, Marsden CD (1989). Mitochondrial complex I deficiency in Parkinson's disease. Lancet, 1(8649), 1269 - .
    • Holt IJ, Harding AE, Cooper JM, Schapira AH, Toscano A, Clark JB, Morgan-Hughes JA (1989). Mitochondrial myopathies: clinical and biochemical features of 30 patients with major deletions of muscle mitochondrial DNA. Ann.Neurol., 26(6), 699 - 708.

    1988

    • Morgan-Hughes JA, Schapira AH, Cooper JM, Clark JB (1988). Molecular defects of NADH-ubiquinone oxidoreductase (complex I) in mitochondrial diseases. J.Bioenerg.Biomembr., 20(3), 365 - 382.
    • Schapira AH, Cooper JM, Morgan-Hughes JA, Patel SD, Cleeter MJ, Ragan CI, Clark JB (1988). Molecular basis of mitochondrial myopathies: polypeptide analysis in complex-I deficiency. Lancet, 1(8584), 500 - 503.
    • Cooper JM, Petty RK, Hayes DJ, Morgan-Hughes JA, Clark JB (1988). Chronic administration of the oral hypoglycaemic agent diphenyleneiodonium to rats. An animal model of impaired oxidative phosphorylation (mitochondrial myopathy). Biochem.Pharmacol., 37(4), 687 - 694.
    • Morgan-Hughes JA, Schapira AH, Cooper JM, Hayes DJ, Clark JB (1988). Human mitochondrial respiratory chain deficiencies. Aust.Paediatr.J., 24 Suppl 1, 55 - 57.
    • Holt IJ, Cooper JM, Morgan-Hughes JA, Harding AE (1988). Deletions of muscle mitochondrial DNA. Lancet, 1(8600), 1462 - .
    • Cooper JM, Petty RK, Hayes DJ, Challiss RA, Brosnan MJ, Shoubridge EA, Radda GK, Morgan-Hughes JA, Clark JB (1988). An animal model of mitochondrial myopathy: a biochemical and physiological investigation of rats treated in vivo with the NADH-CoQ reductase inhibitor, diphenyleneiodonium. J.Neurol.Sci., 83(2-3), 335 - 347.

    1987

    • Morgan-Hughes JA, Cooper JM, Schapira AH, Hayes DJ, Clark JB (1987). The mitochondrial myopathies. Defects of the mitochondrial respiratory chain and oxidative phosphorylation system.. Electroencephalography and Clinical Neurophysiology, Suppl.39, 103 - 114.
    • SCHAPIRA AHV, COOPER JM, MORGANHUGHES JA, LANDON DN (1987). A MITOCHONDRIAL MYOPATHY WITH SPECIFIC DEFICIENCIES OF SUCCINIC-DEHYDROGENASE ACTIVITY AND THE RIESKE IRON SULFUR PROTEIN OF COMPLEX-III. NEUROPATH APPL NEURO, 13(6), 497 - 497.

    1985

    • Morgan-Hughes JA, Hayes DJ, Cooper M, Clark JB (1985). Mitochondrial myopathies: deficiencies localized to complex I and complex III of the mitochondrial respiratory chain.. Biochemical Society Transactions, 13(4), 648 - 650.

    1984

    • Morgan-Hughes JA, Hayes DJ, Clark JB, Cooper JM (1984). Mitochondrial myopathies. Results of exploratory therapeutic trials. In (Ed.), (pp. 417 - 424). : Elsevier/North Holland Biomedical Press.

    • Proukakis C, Dudzik CG, Brier T, McKay DS, Cooper JM, Millhauser GL, Houlden H, Schapira AH (). A novel alpha-synuclein missense mutation in Parkinson's disease. Neurology, , - .