Schöls, Ludger and Zange, Jochen and Abele, M. and Schillings, M. and Skipka, G. and Kuntz-Hehne, S. and van Beekvelt, Mireille C.P. and Colier, Willy N.J.M. and Müller, Klaus and Klockgether, Thomas and Przuntek, Hans and Vorgerd, Matthias (2005) L-carnitine and creatine in Friedreich’s ataxia. A randomized, placebo-controlled crossover trial. Journal of Neural Transmission, 112 (6), pp. 789-796.
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Impaired oxidative phosphorylation is a crucial factor in the pathogenesis of Friedreich’s ataxia (FA). L-carnitine and creatine are natural compounds that can enhance cellular energy transduction. We performed a placebo-controlled triple-phase crossover trial of L-carnitine (3 g=d) and creatine (6.75 g=d) in 16 patients with genetically confirmed FA. Primary outcome measures were mitochondrial ATP production measured as phosphocreatine recovery by <sup>31</sup>Phosphorus magnetic resonance spectroscopy, neurological deficits assessed by the international co-operative ataxia rating scale and cardiac hypertrophy in echocardiography. After 4 months on L-carnitine phosphocreatine recovery was improved compared to baseline (p<0.03, t-test) but comparison to placebo and creatine effects did not reach significance (p<0.06, F-test). Ataxia rating scale and echocardiographic parameters remained unchanged. Creatine had no effect in FA patients. L-carnitine is a promising substance for the treatment of FA patients, and larger trials are warranted.
|Title:||L-carnitine and creatine in Friedreich’s ataxia. A randomized, placebo-controlled crossover trial|
|Journal or Publication Title:||Journal of Neural Transmission|
|In ISI Web of Science:||Yes|
|Page Range:||pp. 789-796|
|Keywords:||Friedreich’s ataxia, therapy, L-carnitine, oxidative phosphorylation, magnetic resonance spectroscopy|
|HGF - Research field:||Aeronautics, Space and Transport (old)|
|HGF - Program:||Space (old)|
|HGF - Program Themes:||W FR - Forschung unter Weltraumbedingungen (old)|
|DLR - Research area:||Space|
|DLR - Program:||W FR - Forschung unter Weltraumbedingungen|
|DLR - Research theme (Project):||W -- no assignement (old)|
|Institutes and Institutions:||Institute of Aerospace Medicine > Space Physiology|
|Deposited By:||Dr.rer.nat. Jochen Zange|
|Deposited On:||10 Jan 2006|
|Last Modified:||27 Apr 2009 04:52|
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