An inhibitor of transforming growth factor beta type I receptor ameliorates muscle atrophy in a mouse model of caveolin 3-deficient muscular dystrophy

Skeletal muscle expressing Pro104Leu mutant caveolin 3 (CAV3P104L) in mouse becomes atrophied and serves as a model of autosomal dominant limb-girdle muscular dystrophy 1C. We previously found that caveolin 3-deficient muscles showed activated intramuscular transforming growth factor beta (TGF-β) si...

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Veröffentlicht in:	Laboratory investigation 2012-08, Vol.92 (8), p.1100-1114
Hauptverfasser:	Ohsawa, Yutaka, Okada, Tadashi, Nishimatsu, Shin-ichiro, Ishizaki, Masatoshi, Suga, Tomohiro, Fujino, Masahiro, Murakami, Tatsufumi, Uchino, Makoto, Tsuchida, Kunihiro, Noji, Sumihare, Hinohara, Atsushi, Shimizu, Toshiyuki, Shimizu, Kiyoshi, Sunada, Yoshihide
Format:	Artikel
Sprache:	eng
Schlagworte:	692/699/375/374 692/700/565/1436/2185 Activin Receptors, Type I - antagonists & inhibitors Activin Receptors, Type I - pharmacology Activins - metabolism Activins - pharmacology Animals Biological and medical sciences Biotechnology caveolin 3 Caveolin 3 - deficiency Caveolin 3 - genetics Caveolin 3 - metabolism Cell Differentiation - drug effects Cell Differentiation - physiology Cell Line Disease Models, Animal Diseases of striated muscles. Neuromuscular diseases Female Fundamental and applied biological sciences. Psychology Gene Expression Regulation HEK293 Cells Humans Investigative techniques, diagnostic techniques (general aspects) Laboratory Medicine limb-girdle muscular dystrophy 1C Male Medical sciences Medicine Medicine & Public Health Mice Mice, Transgenic Muscle Development - drug effects Muscle Fibers, Skeletal - drug effects Muscle Fibers, Skeletal - metabolism muscular atrophy Muscular Dystrophies, Limb-Girdle - drug therapy Muscular Dystrophies, Limb-Girdle - metabolism Muscular Dystrophies, Limb-Girdle - pathology myoblast Myoblasts - pathology Myostatin - metabolism Myostatin - pharmacology Neurology Pathology Protein Serine-Threonine Kinases - antagonists & inhibitors Receptor, Transforming Growth Factor-beta Type I Receptors, Transforming Growth Factor beta - antagonists & inhibitors research-article satellite cell Satellite Cells, Skeletal Muscle - drug effects Signal Transduction small-molecule kinase inhibitor Transforming Growth Factor beta1 - genetics Transforming Growth Factor beta1 - metabolism transforming growth factor-β type I receptor kinase
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