Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration (Articolo in rivista)

Type

• Articolo in rivista (Classe)
• Prodotto della ricerca (Classe)

Label

• Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration (Articolo in rivista) (literal)

Anno

• 2010-01-01T00:00:00+01:00 (literal)

Alternative label

• Grumati P, Coletto L, Sabatelli P, Cescon M, Angelin A, Bertaggia E, Blaauw B, Urciuolo A, Tiepolo T, Merlini L, Maraldi NM, Bernardi P, Sandri M, Bonaldo P. (2010)
  Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration
  in Nature medicine (Print)
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Grumati P, Coletto L, Sabatelli P, Cescon M, Angelin A, Bertaggia E, Blaauw B, Urciuolo A, Tiepolo T, Merlini L, Maraldi NM, Bernardi P, Sandri M, Bonaldo P. (literal)

Pagina inizio

• 1313 (literal)

Pagina fine

• 1320 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 16 (literal)

Rivista

• Nature medicine (Rivista)

Note

• ISI Web of Science (WOS) (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• Department of Histology, Microbiology and Medical Biotechnology, University of Padova, Padova, Italy; Dulbecco Telethon Institute, Venetian Institute of Molecular Medicine, Padova, Italy; Institute of Molecular Genetics—National Research Council, Bologna, Italy; Department of Biomedical Sciences, University of Padova, Padova, Italy; Department of Human Anatomy & Physiology, University of Padova, Padova, Italy; Department of Experimental and Diagnostic Medicine, University of Ferrara, Ferrara, Italy; Department of Anatomical Sciences, University of Bologna, Bologna, Italy. (literal)

Titolo

• Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration (literal)

Abstract

• 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 have previously found that muscular dystrophies linked to collagen VI deficiency show dysfunctional mitochondria and spontaneous apoptosis, leading to myofiber degeneration. Here we demonstrate that this persistence of abnormal organelles and apoptosis are caused by defective autophagy. Skeletal muscles of collagen VI-knockout (Col6a1(-/-)) mice had impaired autophagic flux, which matched the lower induction of beclin-1 and BCL-2/adenovirus E1B-interacting protein-3 (Bnip3) and the lack of autophagosomes after starvation. Forced activation of autophagy by genetic, dietary and pharmacological approaches restored myofiber survival and ameliorated the dystrophic phenotype of Col6a1(-/-) mice. Furthermore, muscle biopsies from subjects with Bethlem myopathy or Ullrich congenital muscular dystrophy had reduced protein amounts of beclin-1 and Bnip3. These findings indicate that defective activation of the autophagic machinery is pathogenic in some congenital muscular dystrophies. (literal)

Prodotto di

• Institute of molecular genetics (IGM) (Istituto)
• Patogenesi delle malattie degenerative muscolo-scheletriche (ME.P05.014.001) (Modulo)

Autore CNR

• NADIR MARIO MARALDI (Unità di personale esterno)
• PATRIZIA SABATELLI (Unità di personale interno)

Incoming links:

Prodotto

• Institute of molecular genetics (IGM) (Istituto)
• Patogenesi delle malattie degenerative muscolo-scheletriche (ME.P05.014.001) (Modulo)

Autore CNR di

• PATRIZIA SABATELLI (Unità di personale interno)
• NADIR MARIO MARALDI (Unità di personale esterno)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• Nature medicine (Rivista)