Thrombospondin expression in myofibers stabilizes muscle membranes.
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ABSTRACT:
SUBMITTER: Vanhoutte D
PROVIDER: S-EPMC5063588 | biostudies-literature | 2016 Sep
REPOSITORIES: biostudies-literature
Publications
Thrombospondin expression in myofibers stabilizes muscle membranes.
Vanhoutte Davy D Schips Tobias G TG Kwong Jennifer Q JQ Davis Jennifer J Tjondrokoesoemo Andoria A Brody Matthew J MJ Sargent Michelle A MA Kanisicak Onur O Yi Hong H Gao Quan Q QQ Rabinowitz Joseph E JE Volk Talila T McNally Elizabeth M EM Molkentin Jeffery D JD
eLife 20160926
Skeletal muscle is highly sensitive to mutations in genes that participate in membrane stability and cellular attachment, which often leads to muscular dystrophy. Here we show that Thrombospondin-4 (Thbs4) regulates skeletal muscle integrity and its susceptibility to muscular dystrophy through organization of membrane attachment complexes. Loss of the <i>Thbs4</i> gene causes spontaneous dystrophic changes with aging and accelerates disease in 2 mouse models of muscular dystrophy, while overexpr ...[more]
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