Volume 53, Issue 1 pp. 121-123
Brief Communications

Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia

Michael Bunse PhD

Corresponding Author

Michael Bunse PhD

Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl

Physikalisches Institut der Universität Tübingen, Tübingen

Hypertension and Diabetes Research Unit, Max Grundig Clinic, Schwarzwaldhochstrasse 1, 77815 Bühl, GermanySearch for more papers by this author
Nana Bit-Avragim MD

Nana Bit-Avragim MD

The Charité, Department of Cardiology, Campus Buch and Campus Virchow clinics, Humboldt University the Max Delbrück Centre for Molecular Medicine, Berlin

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Axel Riefflin MD

Axel Riefflin MD

The KfH, Dialysezentrum, Frankfurt on the Oder

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Andreas Perrot MS

Andreas Perrot MS

The Charité, Department of Cardiology, Campus Buch and Campus Virchow clinics, Humboldt University the Max Delbrück Centre for Molecular Medicine, Berlin

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Oliver Schmidt PhD

Oliver Schmidt PhD

Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl

Physikalisches Institut der Universität Tübingen, Tübingen

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Friedmar R. Kreuz MD

Friedmar R. Kreuz MD

Institute of Clinical Genetics, Medical Faculty Carl Gustav Carus, Technical University, Dresden, Germany

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Rainer Dietz MD

Rainer Dietz MD

The Charité, Department of Cardiology, Campus Buch and Campus Virchow clinics, Humboldt University the Max Delbrück Centre for Molecular Medicine, Berlin

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Wulf-Ingo Jung PhD

Wulf-Ingo Jung PhD

Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl

Physikalisches Institut der Universität Tübingen, Tübingen

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Karl Josef Osterziel MD

Karl Josef Osterziel MD

The Charité, Department of Cardiology, Campus Buch and Campus Virchow clinics, Humboldt University the Max Delbrück Centre for Molecular Medicine, Berlin

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First published: 04 December 2002
Citations: 37

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. Ann Neurol 2003;53:000–000

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