Human Molecular Genetics, Vol 6, 601-607, Copyright © 1997 by Oxford University Press
V Nigro, Y Okazaki, A Belsito, G Piluso, Y Matsuda, L Politano, G Nigro, C Ventura, C Abbondanza, AM Molinari, D Acampora, M Nishimura, Y Hayashizaki and GA Puca
The BIO14.6 hamster is a widely used model for autosomal recessive
cardiomyopathy. These animals die prematurely from progressive myocardial
necrosis and heart failure. The primary genetic defect leading to the
cardiomyopathy is still unknown. Recently, a genetic linkage map localized
the cardiomyopathy locus on hamster chromosome 9qa2.1-b1, excluding several
candidate genes. We now demonstrate that the cardiomyopathy results from a
mutation in the delta-sarcoglycan gene that maps to the disease locus. This
mutation was completely coincident with the disease in backcross and F2
pedigrees. This constitutes the first animal model identified for human
sarcoglycan disorders.
ARTICLES
Identification of the Syrian hamster cardiomyopathy gene
Istituto di Patologia Generale e Oncologia, Facolta di Medicina, Seconda Universita degli Studi di Napoli, Italy.
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