Article
Chimeric protein repair of laminin polymerization ameliorates muscular dystrophy phenotype.
The Journal of clinical investigation - 1 Mar 2017
McKee Karen K, Crosson Stephanie C, Meinen Sarina, Reinhard Judith R, Rüegg Markus A, Yurchenco Peter D
Abstract excerpt
Mutations in laminin α2-subunit (Lmα2, encoded by LAMA2) are linked to approximately 30% of congenital muscular dystrophy cases. Mice with a homozygous mutation in Lama2 (dy2J mice) express a nonpolymerizing form of laminin-211 (Lm211) and are a model for ambulatory-type Lmα2-deficient muscular dystrophy. Here, we developed transgenic dy2J mice with muscle-specific expression of αLNNd, a laminin/nidogen chimeric...
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