Cultured muscle cells display defects of mitochondrial myopathy ameliorated by anti-oxidants. (Articolo in rivista)

Type

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

Label

• Cultured muscle cells display defects of mitochondrial myopathy ameliorated by anti-oxidants. (Articolo in rivista) (literal)

Anno

• 2007-01-01T00:00:00+01:00 (literal)

Alternative label

• Vergani L, Malena A, Sabatelli P, Loro E, Cavallini L, Magalhaes P, Valente L, Bragantini F, Carrara F, Leger B, Poulton J, Russell AP, Holt IJ. (2007)
  Cultured muscle cells display defects of mitochondrial myopathy ameliorated by anti-oxidants.
  in Brain (Print)
  (literal)

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

• Vergani L, Malena A, Sabatelli P, Loro E, Cavallini L, Magalhaes P, Valente L, Bragantini F, Carrara F, Leger B, Poulton J, Russell AP, Holt IJ. (literal)

Pagina inizio

• 2715 (literal)

Pagina fine

• 2724 (literal)

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

• 130 (literal)

Rivista

• Brain (Rivista)

Note

• ISI Web of Science (WOS) (literal)

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

• Department of Neurological Sciences, University of Padova, 35129 Padova; ITOI-CNR IOP, 40136 Bologna; Departments of Biochemistry and Biomedical Sciences, University of Padova, 35121 Padova; Department of Molecular Neurogenetics, National Neurological Institute C.Besta, 20126 Milano, Italy; Clinique Romande de Readaptation, CH-1951 Sion, Switzerland; Department of Obstetrics and Gynaecology, J Radcliffe Hospital, OX3 9DU Oxford, UK,; School of Exercise and Nutrition Sciences, Deakin University, 3125 Australia and 9MRC-Dunn Human Nutrition Unit, University of Cambridge, CB2 OXY Cambridge, UK. (literal)

Titolo

• Cultured muscle cells display defects of mitochondrial myopathy ameliorated by anti-oxidants. (literal)

Abstract

• The mitochondrial DNA A3243G mutation causes neuromuscular disease. To investigate the muscle-specific pathophysiology of mitochondrial disease, rhabdomyosarcoma transmitochondrial hybrid cells (cybrids) were generated that retain the capacity to differentiate to myotubes. In some cases, striated muscle-like fibres were formed after innervation with rat embryonic spinal cord. Myotubes carrying A3243G mtDNA produced more reactive oxygen species than controls, and had altered glutathione homeostasis. Moreover, A3243G mutant myotubes showed evidence of abnormal mitochondrial distribution, which was associated with down-regulation of three genes involved in mitochondrial morphology, Mfn1, Mfn2 and DRP1. Electron microscopy revealed mitochondria with ultrastructural abnormalities and paracrystalline inclusions. All these features were ameliorated by anti-oxidant treatment, with the exception of the paracrystalline inclusions. These data suggest that rhabdomyosarcoma cybrids are a valid cellular model for studying muscle-specific features of mitochondrial disease and that excess reactive oxygen species production is a significant contributor to mitochondrial dysfunction, which is amenable to anti-oxidant therapy. (literal)

Prodotto di

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

Autore CNR

• PATRIZIA SABATELLI (Persona)

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 (Persona)

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

• Brain (Rivista)