title: Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia.
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.
authors.affiliation: Hypertension and Diabetes Research Unit, Max Grundig Clinic, Bühl, Germany. michael.bunse@web.de
authors.forename: Michael
authors.initials: M
authors.lastname: Bunse
authors.forename: Nana
authors.initials: N
authors.lastname: Bit-Avragim
authors.forename: Axel
authors.initials: A
authors.lastname: Riefflin
authors.forename: Andreas
authors.initials: A
authors.lastname: Perrot
authors.forename: Oliver
authors.initials: O
authors.lastname: Schmidt
authors.forename: Friedmar R
authors.initials: FR
authors.lastname: Kreuz
authors.forename: Rainer
authors.initials: R
authors.lastname: Dietz
authors.forename: Wulf-Ingo
authors.initials: WI
authors.lastname: Jung
authors.forename: Karl Josef
authors.initials: KJ
authors.lastname: Osterziel
mesh_terms: 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
chemical_list: D010725:Phosphocreatine; D000255:Adenosine Triphosphate