Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia |
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Authors: | 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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Institution: | Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl, Germany. michael.bunse@web.de |
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Abstract: | 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. |
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