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. 2003 Jan;53(1):121-3.
doi: 10.1002/ana.10419.

Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia

Michael Bunse  1 , Nana Bit-Avragim, Axel Riefflin, Andreas Perrot, Oliver Schmidt, Friedmar R Kreuz, Rainer Dietz, Wulf-Ingo Jung, Karl Josef Osterziel
Affiliations

Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia

Michael Bunse et al. Ann Neurol. 2003 Jan.

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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Comment in

  • Cardiac bioenergetics in Friedreich's ataxia.
    Lodi R, Rajagopalan B, Schapira AH, Cooper JM. Lodi R, et al. Ann Neurol. 2003 Oct;54(4):552; author reply 552-3. doi: 10.1002/ana.10744. Ann Neurol. 2003. PMID: 14520674 No abstract available.

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