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Proc Natl Acad Sci U S A. 2006 Apr 4;103(14):5385-90. Epub 2006 Mar 24.

Cytoplasmic gamma-actin contributes to a compensatory remodeling response in dystrophin-deficient muscle.

Author information

1
Department of Physiology, University of Wisconsin, 1300 University Avenue, Madison, WI 53706, USA.

Abstract

Dystrophin mechanically links the costameric cytoskeleton and sarcolemma, yet dystrophin-deficient muscle exhibits abnormalities in cell signaling, gene expression, and contractile function that are not clearly understood. We generated new antibodies specific for cytoplasmic gamma-actin and confirmed that gamma-actin most predominantly localized to the sarcolemma and in a faint reticular lattice within normal muscle cells. However, we observed that gamma-actin levels were increased 10-fold at the sarcolemma and within the cytoplasm of striated muscle cells from dystrophin-deficient mdx mice. Transgenic overexpression of the dystrophin homologue utrophin, or functional dystrophin constructs in mdx muscle, restored gamma-actin to normal levels, whereas gamma-actin remained elevated in mdx muscle expressing nonfunctional dystrophin constructs. We conclude that increased cytoplasmic gamma-actin in dystrophin-deficient muscle may be a compensatory response to fortify the weakened costameric lattice through recruitment of parallel mechanical linkages. However, the presence of excessive myoplasmic gamma-actin may also contribute to altered cell signaling or gene expression in dystrophin-deficient muscle.

PMID:
16565216
PMCID:
PMC1459364
DOI:
10.1073/pnas.0600980103
[Indexed for MEDLINE]
Free PMC Article

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