Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers. (Articolo in rivista)

Type
Label
  • Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers. (Articolo in rivista) (literal)
Anno
  • 2012-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.4103/0028-3886.103200 (literal)
Alternative label
  • Sabatelli P, Pellegrini C, Faldini C, Merlini L. (2012)
    Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers.
    in Neurology India
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Sabatelli P, Pellegrini C, Faldini C, Merlini L. (literal)
Pagina inizio
  • 510 (literal)
Pagina fine
  • 511 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url
  • http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2012;volume=60;issue=5;spage=510;epage=511;aulast=Sabatelli (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 60 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo
  • 5 (literal)
Note
  • PubMe (literal)
  • ISI Web of Science (WOS) (literal)
  • Scopu (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Institute of Molecular Genetic-CNR, Rizzoli Orthopedic Institute, Bologna, Italy; Department of Rizzoli-Sicily, Rizzoli Orthopedic Institute, Bologna, Italy; Department of Rizzoli-Sicily, Physical Medicine and Rehabilitation, Rizzoli Orthopedic Institute, Bagheria, Italy (literal)
Titolo
  • Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers. (literal)
Abstract
  • In this detailed muscle biopsy study of a patient with molecularly confirmed diagnosis of limb-girdle muscular dystrophy 2I (LGMD2I) we show some new data, that is the presence of altered expression pattern of costamere components as integrin ?7B and integrin ?1D associated with vinculin costameric derangement and basal lamina ultrastructural abnormalities as detachments and discontinuities suggesting that different cellular compartments are involved in LGMD2I and the altered basement membrane-plasmalemma-cytoskeleton binding can underlie muscle degeneration. (literal)
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