Document Detail


Phosphorus-31 magnetic resonance spectroscopy of skeletal muscle in maternally inherited diabetes and deafness A3243G mitochondrial mutation carriers.
MedLine Citation:
PMID:  19097109     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Saskia G C van Elderen; Joost Doornbos; Einar H R van Essen; Herman H P J Lemkes; J Antonie Maassen; Jan W A Smit; Albert de Roos
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of magnetic resonance imaging : JMRI     Volume:  29     ISSN:  1053-1807     ISO Abbreviation:  J Magn Reson Imaging     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2008-12-24     Completed Date:  2009-02-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9105850     Medline TA:  J Magn Reson Imaging     Country:  United States    
Other Details:
Languages:  eng     Pagination:  127-31     Citation Subset:  IM    
Affiliation:
Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. s.g.c.van_elderen@lumc.nl
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MeSH Terms
Descriptor/Qualifier:
Adult
Deafness / physiopathology*
Diabetes Mellitus / genetics,  metabolism,  physiopathology*
Female
Genetic Predisposition to Disease / genetics
Heterozygote
Humans
Magnetic Resonance Spectroscopy
Male
Mitochondria, Muscle / genetics*,  metabolism*
Mitochondrial Diseases / physiopathology*
Mothers
Muscle, Skeletal / metabolism*
Mutation
Phosphorus / analysis*
Phosphorus Isotopes / analysis
Chemical
Reg. No./Substance:
0/Phosphorus Isotopes; 7723-14-0/Phosphorus

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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