Genetic overexpression of Serpina3n attenuates muscular dystrophy in mice

Muscular dystrophy (MD) is associated with mutations in genes that stabilize the myofiber plasma membrane, such as through the dystrophin-glycoprotein complex (DGC). Instability of this complex or defects in membrane repair/integrity leads to calcium influx and myofiber necrosis leading to progressi...

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Veröffentlicht in:	Human molecular genetics 2016-03, Vol.25 (6), p.1192-1202
Hauptverfasser:	Tjondrokoesoemo, Andoria, Schips, Tobias, Kanisicak, Onur, Sargent, Michelle A, Molkentin, Jeffery D
Format:	Artikel
Sprache:	eng
Schlagworte:	Acute-Phase Proteins - biosynthesis Acute-Phase Proteins - genetics Acute-Phase Proteins - metabolism Animals Calcium - metabolism Cell Membrane - metabolism Disease Models, Animal Dystrophin - genetics Dystrophin - metabolism Female Integrins - genetics Integrins - metabolism Male Mice Mice, Inbred C57BL Mice, Inbred mdx Mice, Transgenic Muscle, Skeletal - metabolism Muscular Dystrophy, Animal - genetics Muscular Dystrophy, Animal - metabolism Muscular Dystrophy, Animal - therapy Sarcolemma - metabolism Serpins - biosynthesis Serpins - genetics Serpins - metabolism Transgenes Up-Regulation Utrophin - genetics Utrophin - metabolism
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