Dystroglycan: a possible mediator for reducing congenital muscular dystrophy? (Articolo in rivista)

Type

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

Label

• Dystroglycan: a possible mediator for reducing congenital muscular dystrophy? (Articolo in rivista) (literal)

Anno

• 2007-01-01T00:00:00+01:00 (literal)

Alternative label

• Sciandra F., Gawlik K.I., Brancaccio A., Durbeej M. (2007)
  Dystroglycan: a possible mediator for reducing congenital muscular dystrophy?
  in Trends in biotechnology (Regul. ed.)
  (literal)

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

• Sciandra F., Gawlik K.I., Brancaccio A., Durbeej M. (literal)

Pagina inizio

• 262 (literal)

Pagina fine

• 268 (literal)

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

• 25 (literal)

Rivista

• Trends in biotechnology (Rivista)

Note

• Scopu (literal)
• ISI Web of Science (WOS) (literal)
• PubMe (literal)

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

• Gawlik K.I., Durbeej M.: University of Lund; Sciandra F., Brancaccio A.: ICRM-CNR (literal)

Titolo

• Dystroglycan: a possible mediator for reducing congenital muscular dystrophy? (literal)

Abstract

• alpha-Dystroglycan is a highly glycosylated peripheral protein forming a complex with the membrane-spanning beta-dystroglycan and establishing a connection between the extracellular matrix and the cytoskeleton. In skeletal muscle, as part of the larger dystrophin-glycoprotein complex, dystroglycan is believed to be essential for maintaining the structural and functional stability of muscle fibers. Recent work highlights the role of abnormal dystroglycan glycosylation at the basis of glycosyltransferase- deficient congenital muscular dystrophies. Notably, modulation of glycosyltransferase activity can restore a-dystroglycan receptor function in these disorders. Moreover, transgenic approaches favoring the interaction between dystroglycan and the extracellular matrix molecules also represent an innovative way to restore skeletal muscle structure. These pioneering approaches might comprise an important first step towards the design of gene-transfer-based strategies for the rescue of congenital muscular dystrophies involving dystroglycan. (literal)

Prodotto di

• Modellistica molecolare di proteine legate a processi patologici (PM.P01.026.002) (Modulo)
• Istituto di chimica del riconoscimento molecolare (ICRM) (Istituto)

Autore CNR

• FRANCESCA SCIANDRA (Unità di personale esterno)
• ANDREA BRANCACCIO (Unità di personale interno)

Incoming links:

Autore CNR di

• ANDREA BRANCACCIO (Unità di personale interno)
• FRANCESCA SCIANDRA (Unità di personale esterno)

Prodotto

• Istituto di chimica del riconoscimento molecolare (ICRM) (Istituto)
• Modellistica molecolare di proteine legate a processi patologici (PM.P01.026.002) (Modulo)

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

• Trends in biotechnology (Rivista)