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Phosphorus-31 magnetic resonance spectroscopy of skeletal muscle in maternally inherited diabetes and deafness A3243G mitochondrial mutation carriers.
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.
Afiliación
  • van Elderen SG; Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. s.g.c.van_elderen@lumc.nl
J Magn Reson Imaging ; 29(1): 127-31, 2009 Jan.
Article en En | MEDLINE | ID: mdl-19097109
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fósforo / Músculo Esquelético / Enfermedades Mitocondriales / Sordera / Diabetes Mellitus / Mitocondrias Musculares Límite: Adult / Female / Humans / Male Idioma: En Revista: J Magn Reson Imaging Asunto de la revista: DIAGNOSTICO POR IMAGEM Año: 2009 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fósforo / Músculo Esquelético / Enfermedades Mitocondriales / Sordera / Diabetes Mellitus / Mitocondrias Musculares Límite: Adult / Female / Humans / Male Idioma: En Revista: J Magn Reson Imaging Asunto de la revista: DIAGNOSTICO POR IMAGEM Año: 2009 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Estados Unidos