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Phosphorus‐31 magnetic resonance spectroscopy of skeletal muscle in maternally inherited diabetes and deafness A3243G mitochondrial mutation carriers
Author(s) -
van Elderen Saskia G.C.,
Doornbos Joost,
van Essen Einar H.R.,
Lemkes Herman H.P.J.,
Maassen J. Antonie,
Smit Jan W.A.,
de Roos Albert
Publication year - 2009
Publication title -
journal of magnetic resonance imaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.563
H-Index - 160
eISSN - 1522-2586
pISSN - 1053-1807
DOI - 10.1002/jmri.21620
Subject(s) - phosphocreatine , inorganic phosphate , skeletal muscle , diabetes mellitus , medicine , endocrinology , mitochondrial dna , magnetic resonance imaging , pi , calf muscle , mutation , phosphate , energy metabolism , biology , gene , genetics , biochemistry , radiology
Purpose To investigate high‐energy phosphate metabolism in striated skeletal muscle of patients with Maternally Inherited Diabetes and Deafness (MIDD) syndrome. Materials and Methods In 11 patients with the MIDD mutation (six with diabetes mellitus [DM] and five non‐DM) and eight healthy subjects, phosphocreatine (PCr) and inorganic phosphate (Pi) in the vastus medialis muscle was measured immediately after exercise using 31 P‐magnetic resonance spectroscopy (MRS). The half‐time of recovery (t1/2) of monoexponentially fitted (PCr+Pi)/PCr was calculated from spectra obtained every 4 seconds after cessation of exercise. A multiple linear regression model was used for statistical analysis. Results Patients with the MIDD mutation showed a significantly prolonged t1/2 (PCr+Pi)/PCr after exercise as compared to controls (13.6±3.0 vs. 8.7±1.3 sec, P = 0.01). No association between the presence of DM and t1/2 (PCr + Pi)/PCr was found ( P = 0.382). Conclusion MIDD patients showed impaired mitochondrial oxidative phosphorylation in skeletal muscle shortly after exercise, irrespective of the presence of DM. J. Magn. Reson. Imaging 2009;29:127–131. © 2008 Wiley‐Liss, Inc.