A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin.
Neuromuscul Disord
; 12(3): 247-57, 2002 Mar.
Article
en En
| MEDLINE
| ID: mdl-11801396
Muscle energetics and function were investigated in the hindlimb of mice lacking dystrophin (mdx), utrophin and dystrophin (utr-dys) and controls (C57Bl/10) using 31P and 1H magnetic resonance techniques, electrical nerve stimulation and direct biochemical analysis. At rest, [adenosine triphosphate] and [total creatine] were lowest in utr-dys, while [inorganic phosphate] was elevated. Calculated [adenosine diphosphate] was 3-fold higher in mdx and 5-fold higher in utr-dys than in controls, consistent with an increased adenosine triphosphate requirement for ion pump activity. During stimulation, force production was low only in utr-dys, and this was reflected in the bioenergetic changes. Initial recovery rates of [phosphocreatine] and [adenosine diphosphate] after stimulation were rapid in all groups, indicative of normal mitochondrial adenosine triphosphate production in utr-dys and mdx. Recovery of pH was slow in utr-dys. The data indicate that the severe abnormalities which are present in the absence of utrophin and dystrophin leave basic muscle energetics intact and appear confined to processes involving the sarcolemma.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Distrofina
/
Músculo Esquelético
/
Proteínas del Citoesqueleto
/
Metabolismo Energético
/
Proteínas de la Membrana
/
Distrofia Muscular Animal
Límite:
Animals
Idioma:
En
Revista:
Neuromuscul Disord
Asunto de la revista:
NEUROLOGIA
Año:
2002
Tipo del documento:
Article
Pais de publicación:
Reino Unido