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PMID: 8020621 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Review

Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles.

Experientia ·Vol. 50 ·No. 6 ·1994-06-15 ·Pages 602-5

Chinet AE, Even PC, Decrouy A

Abstract

Muscles from the mdx mouse (X-linked genetic disorder similar to Duchenne muscular dystrophy) lack dystrophin-associated transsarcolemmal proteins and show reduced maintenance metabolic rates. Here, microcalorimetric comparisons of metabolic stimulation by exogenous substrates in isolated muscles revealed substrate-selective limitation of chemical reaction rates through both glycolytic and TCA-cycle pathways, identical in slow- and fast-twitch mdx muscles. This systemic approach, as opposed to comparisons of single-enzyme activities, sheds new light on the function of dystrophin and associated proteins. The in vivo efficiency of metabolic pathways may depend on stabilization of enzyme complexes by dystrophin-associated elements of the cytoskeleton.

MeSH Terms
Animals Citric Acid Cycle Cytoskeleton/metabolism Dystrophin/physiology Energy Metabolism Glycolysis Mice Mice, Mutant Strains Muscles/metabolism Muscular Dystrophy, Animal/metabolism
Chemicals
Dystrophin
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Chinet A E
Département de Physiologie, Centre Médical Universitaire, Geneva, Switzerland.
Even P C
Decrouy A
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Article Info
Journal
Experientia
Abbr.
Experientia
ISSN
0014-4754
DOI
10.1007/BF01921731
Published
1994-06-15
Pages
602-5
Language
English
Region
Switzerland
NLM ID
0376547
Subset
IM
External Links
PubMed source DOI: 10.1007/BF01921731
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