Changes in Muscle Cell Metabolism and Mechanotransduction Are Associated with Myopathic Phenotype in a Mouse Model of Collagen VI Deficiency. (Articolo in rivista)

Type

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

Label

• Changes in Muscle Cell Metabolism and Mechanotransduction Are Associated with Myopathic Phenotype in a Mouse Model of Collagen VI Deficiency. (Articolo in rivista) (literal)

Anno

• 2013-01-01T00:00:00+01:00 (literal)

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

• 10.1371/journal.pone.0056716 (literal)

Alternative label

• Sara De Palma; Roberta Leone; Paolo Grumati; Michele Vasso; Roman Polishchuk; Daniele Capitanio; Paola Braghetta; Paolo Bernardi; Paolo Bonaldo; and Cecilia Gelfi (2013)
  Changes in Muscle Cell Metabolism and Mechanotransduction Are Associated with Myopathic Phenotype in a Mouse Model of Collagen VI Deficiency.
  in PloS one
  (literal)

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

• Sara De Palma; Roberta Leone; Paolo Grumati; Michele Vasso; Roman Polishchuk; Daniele Capitanio; Paola Braghetta; Paolo Bernardi; Paolo Bonaldo; and Cecilia Gelfi (literal)

Pagina inizio

• e56716 (literal)

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

• 8 (literal)

Rivista

• PloS one (Rivista)

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

• 2 (literal)

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

• Department of Biomedical Sciences for Health, University of Milan, Segrate (MI), Italy. Institute of Molecular Bioimaging and Physiology, National Research Council (CNR), Segrate (MI), Italy. Department of Biomedical Sciences, University of Padova, Padova, Italy. Telethon Institute of Genetics and Medicine, Institute of Protein Biochemistry, Italian National Research Council (CNR), Naples, Italy. University of Michigan, United States of America. (literal)

Titolo

• Changes in Muscle Cell Metabolism and Mechanotransduction Are Associated with Myopathic Phenotype in a Mouse Model of Collagen VI Deficiency. (literal)

Abstract

• This study identifies metabolic and protein phenotypic alterations in gastrocnemius, tibialis anterior and diaphragm muscles of Col6a1-/- mice, a model of human collagen VI myopathies. All three muscles of Col6a1-/- mice show some common changes in proteins involved in metabolism, resulting in decreased glycolysis and in changes of the TCA cycle fluxes. These changes lead to a different fate of ?-ketoglutarate, with production of anabolic substrates in gastrocnemius and tibialis anterior, and with lipotoxicity in diaphragm. The metabolic changes are associated with changes of proteins involved in mechanotransduction at the myotendineous junction/costameric/sarcomeric level (TN-C, FAK, ROCK1, troponin I fast) and in energy metabolism (aldolase, enolase 3, triose phosphate isomerase, creatine kinase, adenylate kinase 1, parvalbumin, IDH1 and FASN). Together, these change may explain Ca2+ deregulation, impaired force development, increased muscle-relaxation-time and fiber damage found in the mouse model as well as in patients. The severity of these changes differs in the three muscles (gastrocnemius

Prodotto di

• Institute of molecular bioimaging and physiology (IBFM) (Istituto)
• Proteomica differenziale di tessuti patologici e fluidi biologici per la comprensione della biologia dei sistemi (ME.P06.026.002) (Modulo)

Autore CNR

• MICHELE VASSO (Unità di personale interno)
• CECILIA GELFI (Unità di personale esterno)
• SARA DE PALMA (Persona)

Incoming links:

Prodotto

• Institute of molecular bioimaging and physiology (IBFM) (Istituto)
• Proteomica differenziale di tessuti patologici e fluidi biologici per la comprensione della biologia dei sistemi (ME.P06.026.002) (Modulo)

Autore CNR di

• SARA DE PALMA (Persona)
• MICHELE VASSO (Unità di personale interno)
• CECILIA GELFI (Unità di personale esterno)

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

• PloS one (Rivista)