title: Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration.
authors.affiliation: Department of Histology, Microbiology & Medical Biotechnology, University of Padova, Padova, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Grumati
authors.forename: Luisa
authors.initials: L
authors.lastname: Coletto
authors.forename: Patrizia
authors.initials: P
authors.lastname: Sabatelli
authors.forename: Matilde
authors.initials: M
authors.lastname: Cescon
authors.forename: Alessia
authors.initials: A
authors.lastname: Angelin
authors.forename: Enrico
authors.initials: E
authors.lastname: Bertaggia
authors.forename: Bert
authors.initials: B
authors.lastname: Blaauw
authors.forename: Anna
authors.initials: A
authors.lastname: Urciuolo
authors.forename: Tania
authors.initials: T
authors.lastname: Tiepolo
authors.forename: Luciano
authors.initials: L
authors.lastname: Merlini
authors.forename: Nadir M
authors.initials: NM
authors.lastname: Maraldi
authors.forename: Paolo
authors.initials: P
authors.lastname: Bernardi
authors.forename: Marco
authors.initials: M
authors.lastname: Sandri
authors.forename: Paolo
authors.initials: P
authors.lastname: Bonaldo
mesh_terms: D000818:Animals; D017209:Apoptosis; D051017:Apoptosis Regulatory Proteins / Q000378:metabolism; D001343:Autophagy; D000071186:Beclin-1; D015153:Blotting, Western; D002467:Cell Nucleus / Q000378:metabolism; D024142:Collagen Type VI / Q000172:deficiency / Q000378:metabolism*; D003964:Diaphragm / Q000473:pathology / Q000648:ultrastructure; D006801:Humans; D020287:In Situ Nick-End Labeling; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL; D024101:Mitochondrial Proteins / Q000378:metabolism; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology*; D009136:Muscular Dystrophies / Q000378:metabolism / Q000473:pathology*; D010641:Phenotype
chemical_list: D051017:Apoptosis Regulatory Proteins; C508686:BNip3 protein, mouse; D000071186:Beclin-1; C115010:Becn1 protein, mouse; C553839:Col6a1 protein, human; C540085:Col6a1 protein, mouse; D024142:Collagen Type VI; D008565:Membrane Proteins; D024101:Mitochondrial Proteins