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Abstract #0686

Metabolic Adaptations of Creatine Deficiency in Skeletal Muscle

Christine Nabuurs1, Martijn Romeijn1, Andor Veltien1, Hermien Kan1, Dirk Isbrandt2, Arend Heerschap1

1Radiology, Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; 2Center for molecular neurobiology, Institute for signal transmission, Hamburg, Germany

Defects in the biosynthesis of Cr are known to have major effects on brain function and can be successfully treated by Cr supplementation. However, effects of Cr on muscle metabolism have not been fully elucidated yet. In this study, in vivo 31P MRS was applied to yield responses of PCr and Pi levels to Cr supplementation and ischemic conditions in knockout mice with a defect in Cr biosynthesis. The results indicate an upregulation of ATPase at very low PCr concentrations and changes in the PCrATPPi equilibrium during treatment and restriction of Cr.