Unknown

Dataset Information

0

Skeletal muscle-specific ablation of gamma(cyto)-actin does not exacerbate the mdx phenotype.


ABSTRACT: We previously documented a ten-fold increase in gamma(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased gamma(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To explore whether increased gamma(cyto)-actin fortifies the cortical cytoskeleton in dystrophic skeletal muscle, we generated double knockout mice lacking both dystrophin and gamma(cyto)-actin specifically in skeletal muscle (ms-DKO). Surprisingly, dystrophin-deficient mdx and ms-DKO mice presented with comparable levels of myofiber necrosis, membrane instability, and deficits in muscle function. The lack of an exacerbated phenotype in ms-DKO mice suggests gamma(cyto)-actin and dystrophin function in a common pathway. Finally, because both mdx and ms-DKO skeletal muscle showed similar levels of utrophin expression and presented with identical dystrophies, we conclude utrophin can partially compensate for the loss of dystrophin independent of a gamma(cyto)-actin-utrophin interaction.

SUBMITTER: Prins KW 

PROVIDER: S-EPMC2409075 | biostudies-literature | 2008 Jun

REPOSITORIES: biostudies-literature

altmetric image

Publications

Skeletal muscle-specific ablation of gamma(cyto)-actin does not exacerbate the mdx phenotype.

Prins Kurt W KW   Lowe Dawn A DA   Ervasti James M JM  

PloS one 20080611 6


We previously documented a ten-fold increase in gamma(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased gamma(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To explore whether increased gamma(cyto)-actin fortifies the cortical cytoskeleton in dystrophic skeletal muscle, we generated double knockout mice lacking both dystrophin and gamma(cyto)-actin specifically in skeletal muscle (ms-DKO). Surprisingly, dystrop  ...[more]

Similar Datasets

| S-EPMC3048892 | biostudies-other
| S-EPMC5349784 | biostudies-literature
2009-05-24 | E-GEOD-12882 | biostudies-arrayexpress
| S-EPMC3267970 | biostudies-literature
2009-05-25 | GSE12882 | GEO
2018-10-22 | PXD009680 | Pride
| S-EPMC5988407 | biostudies-literature
| S-EPMC1850802 | biostudies-literature
| S-EPMC4168821 | biostudies-other
| S-EPMC5596936 | biostudies-literature