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Article

Spontaneous muscular dystrophy caused by a retrotransposal insertion in the mouse laminin alpha2 chain gene.

Neuromuscular disorders : NMD - 1 Mar 2003

Besse Sylvie, Allamand Valérie, Vilquin Jean-Thomas, Li Zhenlin, Poirier Christophe, Vignier Nicolas, Hori Hisae, Guénet Jean-Louis, Guicheney Pascale

Abstract excerpt

We identified a novel spontaneous mouse model of human congenital muscular dystrophy with laminin alpha2 chain deficiency, named dy(Pas)/dy(Pas). Homozygous animals rapidly developed a progressive muscular dystrophy leading to premature death. Immunohistological and biochemical analyses demonstrated the absence of laminin alpha2 chain expression in skeletal muscle. Analysis of the laminin alpha2 chain cDNA showed...

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Identifiers

PMID: 12609503
DOI: 10.1016/s0960-8966(02)00278-x

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