P
Paul E. Hart
Researcher at University College London
Publications - 16
Citations - 1175
Paul E. Hart is an academic researcher from University College London. The author has contributed to research in topics: Ataxia & Frataxin. The author has an hindex of 8, co-authored 15 publications receiving 1117 citations. Previous affiliations of Paul E. Hart include Royal Free Hospital.
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Journal ArticleDOI
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.
Sarah J. Tabrizi,J Workman,Paul E. Hart,L Mangiarini,Amarbirpal Mahal,Gillian P. Bates,JM Cooper,Anthony H.V. Schapira +7 more
TL;DR: A transgenic mouse model (R6/2) of Huntington's disease developed a movement disorder, muscle wasting, and premature death at about 14 to 16 weeks, with selective neuronal death in these mice is not seen until 14 weeks.
Journal ArticleDOI
Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia
R. Lodi,R. Lodi,Paul E. Hart,Bheeshma Rajagopalan,Doris J. Taylor,Jenifer G. Crilley,J.L. Bradley,Andrew M. Blamire,David Neil Manners,Peter Styles,Anthony H.V. Schapira,J. Mark Cooper +11 more
TL;DR: Partial reversal of a surrogate biochemical marker in FA with antioxidant therapy is demonstrated and supports the evaluation of such therapy as a disease‐modifying strategy in this neurodegenerative disorder.
Journal ArticleDOI
Antioxidant treatment of patients with Friedreich ataxia : four-year follow-up
Paul E. Hart,Raffaele Lodi,Bheeshma Rajagopalan,J.L. Bradley,Jenifer G. Crilley,Chris Turner,Andrew M. Blamire,David Neil Manners,Peter Styles,Anthony H.V. Schapira,J. Mark Cooper +10 more
TL;DR: This therapy resulted in sustained improvement in mitochondrial energy synthesis that was associated with a slowing of the progression of certain clinical features and a significant improvement in cardiac function.
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Coenzyme Q10 and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy
TL;DR: High dose coenzyme Q10 (CoQ10)/vitamin E therapy in Friedreich’s ataxia patients resulted in significant clinical improvements in most patients and the potential for this treatment to modify clinical progression in FRDA was investigated in a randomized double blind trial.
Journal ArticleDOI
Role of oxidative damage in Friedreich's ataxia.
TL;DR: It is proposed that abnormal respiratory chain function and iron accumulation may lead to a progressive increase in oxidative damage, but increased sensitivity to free radicals may not require detectable respiratory chain dysfunction.