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My scientific training was in the field of mitochondrial biochemistry in disease under the supervision of John Morgan-Hughes and John Clark (PhD 1987) at the Institute of Neurology, London. I characterisation the defects in patients with mitochondrial disorders which led to the identification of the first mtDNA deletions (Holt et al 1988, 1989),
In 1990 I was appointed as Lecturer in the Department of Protein and Molecular Biology at the Royal Free Hospital Medical School, and subsequently in 1994 as Senior Lecturer in the department of Clinical Neurosciences. In 2010 I was appointed as Reader in Neurodegenerative diseases in the department of Clinical Neurosciences UCL Institute of Neurology.
Mitochondrial function continued to play an important role in my research, studying Leber’s hereditary Optic Neuropathy (Cock et al 1995) and various other mtDNA mutations (Morten et al 1993) and demonstrated mtDNA depletion syndrome was a nuclear gene disorder (Bodnar et al 1993). I generated an in vivo model to recapitulate the biochemical phenotypes (Cooper et al 1988) and therapeutic evaluation (Cooper et al 1992).
Mitochondrial function in neurodegenerative diseases continues to play a larg part of my research covering ;PD (Schapira et al 1989), Alzheimer’s disease (Cooper et al 1993), HD (Gu et al 1996), Friedreich's ataxia (Bradley et al 2000), Wilson’s disease (Gu et al 2000), ageing (Cooper et al 1992) ;oxidative stress (Thomas et al 1993, Rafique et al 2001) and increased iron (Hartley et al 1993).
Friedreich’s Ataxia (FRDA) research made up a significant part of my work.We confirmed the role of mitochondrial dysfunction, iron accumulation (Bradley et al 2000) and oxidative damage (Bradley et al 2004) in patients with FRDA. Using 31P MRS in collaboration with Raphaele Lodi we were able to detect the mitochondrial ox phos defects in vivo in skeletal muscle (Lodi et al 1999) and cardiac muscle (Lodi et al 2001). We obtained extensive clinical data on over 70 FRDA patients involving neurological, cardiac (Rajagopalan et al 2010), speech and limb co-ordination evaluations and evaluation of quality of life and activities of daily living for the cross sectional analysis of the natural history of FRDA (Cooper et al 2008). Long term vitamin E and coenzyme Q10 therapy trials identified an improvement in mitochondrial function (Lodi et al 2001, Hart et al 2005) and clinical scores (Cooper et al 2008). We have evaluated the ICARS scale for the evaluation of FRDA (Cano et al 2005, Metz et al 2013) and generated a new validated patient scale – Friedreich’s Ataxia Impact Scale (FAIS) (Cano et al 2009). I have collaborated with Mark Pook in the analysis of a transgenic mouse model (Pook et al 2001, Al-Mahdawi et al 2006).
In 1990 I was appointed as Lecturer in the Department of Protein and Molecular Biology at the Royal Free Hospital Medical School, and subsequently in 1994 as Senior Lecturer in the department of Clinical Neurosciences. In 2010 I was appointed as Reader in Neurodegenerative diseases in the department of Clinical Neurosciences UCL Institute of Neurology.
Mitochondrial function continued to play an important role in my research, studying Leber’s hereditary Optic Neuropathy (Cock et al 1995) and various other mtDNA mutations (Morten et al 1993) and demonstrated mtDNA depletion syndrome was a nuclear gene disorder (Bodnar et al 1993). I generated an in vivo model to recapitulate the biochemical phenotypes (Cooper et al 1988) and therapeutic evaluation (Cooper et al 1992).
Mitochondrial function in neurodegenerative diseases continues to play a larg part of my research covering ;PD (Schapira et al 1989), Alzheimer’s disease (Cooper et al 1993), HD (Gu et al 1996), Friedreich's ataxia (Bradley et al 2000), Wilson’s disease (Gu et al 2000), ageing (Cooper et al 1992) ;oxidative stress (Thomas et al 1993, Rafique et al 2001) and increased iron (Hartley et al 1993).
Friedreich’s Ataxia (FRDA) research made up a significant part of my work.We confirmed the role of mitochondrial dysfunction, iron accumulation (Bradley et al 2000) and oxidative damage (Bradley et al 2004) in patients with FRDA. Using 31P MRS in collaboration with Raphaele Lodi we were able to detect the mitochondrial ox phos defects in vivo in skeletal muscle (Lodi et al 1999) and cardiac muscle (Lodi et al 2001). We obtained extensive clinical data on over 70 FRDA patients involving neurological, cardiac (Rajagopalan et al 2010), speech and limb co-ordination evaluations and evaluation of quality of life and activities of daily living for the cross sectional analysis of the natural history of FRDA (Cooper et al 2008). Long term vitamin E and coenzyme Q10 therapy trials identified an improvement in mitochondrial function (Lodi et al 2001, Hart et al 2005) and clinical scores (Cooper et al 2008). We have evaluated the ICARS scale for the evaluation of FRDA (Cano et al 2005, Metz et al 2013) and generated a new validated patient scale – Friedreich’s Ataxia Impact Scale (FAIS) (Cano et al 2009). I have collaborated with Mark Pook in the analysis of a transgenic mouse model (Pook et al 2001, Al-Mahdawi et al 2006).
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María Izco,Ariane Vettorazzi, Raquel Forcen,Javier Blesa,Maria de Toro, Natalia Alvarez-Herrera,J Mark Cooper,Elena Gonzalez-Peñas,Adela Lopez de Cerain,Lydia Alvarez-Erviti
Food and chemical toxicology : an international journal published for the British Industrial Biological Research Association (2021): 112164-112164
Cell Death & Diseaseno. 3 (2013): e545-e545
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P E Hart,Raffaele Lodi,B Rajagopalan,J L Bradley, J G Crilley,Christopher J Turner,Andrew M Blamire, D Manners,Peter Styles,Anthony H V Schapira,J Mark Cooper
mag(2005)
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