title: RhoA leads to up-regulation and relocalization of utrophin in muscle fibers.
abstract: Up-regulation of utrophin, a homolog of dystrophin, is known to ameliorate the dystrophic phenotype in animal models of Duchenne muscular dystrophy. We have previously demonstrated that the active form of RhoA (RhoAV14) increases the expression of utrophin and its localization at the plasma membrane in cultured myoblasts. In this paper, we ask whether RhoAV14 can up-regulate utrophin also in mice. A plasmid encoding for RhoAV14 was injected into skeletal muscles followed by electroporation. Muscles expressing RhoAV14 were analyzed by Western-immunoblotting, real time PCR amplification and immunohistochemistry. We found that RhoAV14 increased utrophin protein expression and distribution specifically at the plasma membrane in muscle fibers without any effect on utrophin transcription. Utrophin up-regulation, uncoupled from that of its mRNA, has been previously observed in pathological processes and in normal regenerating conditions.
authors.affiliation: CRBM, Universités Montpellier 2 et 1, CNRS, 1919 Route de Mende, 34293 Montpellier, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Gauthier-Rouvière
authors.forename: Armelle
authors.initials: A
authors.lastname: Bonet-Kerrache
mesh_terms: D000818:Animals; D002462:Cell Membrane / Q000378:metabolism; D018274:Electroporation; D051379:Mice; D008810:Mice, Inbred C57BL; D018485:Muscle Fibers, Skeletal / Q000378:metabolism*; D020388:Muscular Dystrophy, Duchenne / Q000628:therapy; D014162:Transfection; D015854:Up-Regulation; D049411:Utrophin / Q000235:genetics / Q000378:metabolism*; D020741:rho GTP-Binding Proteins / Q000235:genetics / Q000502:physiology*; D020742:rhoA GTP-Binding Protein
chemical_list: D049411:Utrophin; C526380:RhoA protein, mouse; D020741:rho GTP-Binding Proteins; D020742:rhoA GTP-Binding Protein