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 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.
Bunse
Michael
M
Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl, Germany. michael.bunse@web.de
Bit-Avragim
Nana
N
Riefflin
Axel
A
Perrot
Andreas
A
Schmidt
Oliver
O
Kreuz
Friedmar R
FR
Dietz
Rainer
R
Jung
Wulf-Ingo
WI
Osterziel
Karl Josef
KJ
D000255:Adenosine Triphosphate / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D006332:Cardiomegaly / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D004734:Energy Metabolism; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications* / Q000378:metabolism*; D006346:Heart Septum / Q000473:pathology; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D010725:Phosphocreatine / Q000378:metabolism
D010725:Phosphocreatine; D000255:Adenosine Triphosphate