Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration.
Grumati
Paolo
P
Department of Histology, Microbiology & Medical Biotechnology, University of Padova, Padova, Italy.
Coletto
Luisa
L
Sabatelli
Patrizia
P
Cescon
Matilde
M
Angelin
Alessia
A
Bertaggia
Enrico
E
Blaauw
Bert
B
Urciuolo
Anna
A
Tiepolo
Tania
T
Merlini
Luciano
L
Maraldi
Nadir M
NM
Bernardi
Paolo
P
Sandri
Marco
M
Bonaldo
Paolo
P
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
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