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The absence of dystrophin brain isoform expression in healthy human heart ventricles explains the pathogenesis of 5' X-linked dilated cardiomyopathy

BACKGROUND: In X-linked dilated cardiomyopathy due to dystrophin mutations which abolish the expression of the M isoform (5'-XLDC), the skeletal muscle is spared through the up-regulation of the Brain (B) isoform, a compensatory mechanism that does not appear to occur in the heart of affected i...

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Detalles Bibliográficos
Autores principales: Neri, Marcella, Valli, Emanuele, Alfano, Giovanna, Bovolenta, Matteo, Spitali, Pietro, Rapezzi, Claudio, Muntoni, Francesco, Banfi, Sandro, Perini, Giovanni, Gualandi, Francesca, Ferlini, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3331845/
https://www.ncbi.nlm.nih.gov/pubmed/22455600
http://dx.doi.org/10.1186/1471-2350-13-20