Article
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration.
Nature medicine - 1 Nov 2010
Grumati Paolo, Coletto Luisa, Sabatelli Patrizia, Cescon Matilde, Angelin Alessia, Bertaggia Enrico, Blaauw Bert, Urciuolo Anna, Tiepolo Tania, Merlini Luciano, Maraldi Nadir M, Bernardi Paolo, Sandri Marco, Bonaldo Paolo
Abstract excerpt
Autophagy is crucial in the turnover of cell components, and clearance of damaged organelles by the autophagic-lysosomal pathway is essential for tissue homeostasis. Defects of this degradative system have a role in various diseases, but little is known about autophagy in muscular dystrophies. We...
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