Marked reduction of focal adhesion kinase, serum response factor and myocyte enhancer factor 2C, but increase in RhoA and myostatin in the hindlimb dy mouse muscles

Acta Neuropathologica
Kunihiro SakumaMasahiro Yasuhara

Abstract

Laminin alpha2 (merosin)-deficient congenital muscular dystrophy (CMD) patients show progressive muscle fiber necrosis and ineffective muscle regeneration. This is probably due to decreased formation of multi nucleated myotubes resulting from a myoblast fusion defect. When receiving a mechanical signal from muscle membranes, a cascade of RhoA, focal adhesion kinase (FAK), and serum response factor (SRF) positively regulates myogenesis and muscle hypertrophy associated with functional overload. In contrast, myostatin, a potent negative regulator of skeletal muscle hypertrophy, appears to be up-regulated in the muscles of mdx mice, an animal model for Duchenne muscular dystrophy. Using Western blot and immunohistochemical analyses, we investigated the levels of RhoA, FAK, SRF, and myostatin in the skeletal muscles of dy mice. The amount of RhoA protein was increased in the hindlimb muscles of dy mice aged 12 weeks. At 12 weeks, FAK immunoreactivity was observed in the myonuclei and/or satellite cells of normal mice, but not of dy mice. SRF protein levels decreased markedly in the gastrocnemius and rectus femoris muscles of dy mice at 2 and 12 weeks. Several muscle fibers in normal mice possessed uniform SRF immunoreactivity in th...Continue Reading

Citations

Jan 15, 2008·Pflügers Archiv : European journal of physiology·François B FavierDamien Freyssenet
Dec 17, 2008·PloS One·Charlotte LahouteDominique Daegelen
Jul 11, 2013·PloS One·Tejaswini Narayanan, Shankar Subramaniam
Jul 5, 2015·Journal of Muscle Research and Cell Motility·Zachary A GrahamChristopher P Cardozo
Sep 16, 2014·Frontiers in Aging Neuroscience·Kunihiro SakumaAkihiko Yamaguchi
Aug 29, 2016·Translational Oncology·Alicia M WatersElizabeth A Beierle
May 26, 2010·Laboratory Investigation; a Journal of Technical Methods and Pathology·Joseph M Miano
May 12, 2019·Cells·Lisbeth Liliendal Valbjørn MøllerLykke Sylow
Dec 12, 2019·Neural Regeneration Research·Ana C CalvoRosario Osta
Jun 21, 2005·The Biochemical Journal·Armelle Bonet-KerracheCécile Gauthier-Rouvière

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