Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia

Friedreich's ataxia is a neurodegenerative disease frequently associated with hypertrophic cardiomyopathy. We have determined mitochondrial ATP, phosphocreatine, and intracellular inorganic phosphate levels by 31P nuclear magnetic resonance spectroscopy in the heart of 11 Friedreich's atax...

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Veröffentlicht in:Annals of neurology 2003-01, Vol.53 (1), p.121-123
Hauptverfasser: Bunse, Michael, Bit-Avragim, Nana, Riefflin, Axel, Perrot, Andreas, Schmidt, Oliver, Kreuz, Friedmar R., Dietz, Rainer, Jung, Wulf-Ingo, Osterziel, Karl Josef
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Sprache:eng
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Zusammenfassung:Friedreich's ataxia is a neurodegenerative disease frequently associated with hypertrophic cardiomyopathy. We have determined mitochondrial ATP, phosphocreatine, and intracellular inorganic phosphate levels by 31P nuclear magnetic resonance spectroscopy in the heart of 11 Friedreich's ataxia patients and 11 healthy controls. For the first time, to our knowledge, we showed a significant correlation between the extent of myocardial energy deficiency and the degree of myocardial hypertrophy. When combining our results with previous works on Friedreich's ataxia, these novel findings suggest that energy metabolism is most likely the cause and hypertrophy the effect in Friedreich's ataxia. Ann Neurol 2003;53:000–000
ISSN:0364-5134
1531-8249
DOI:10.1002/ana.10419