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Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus
Journal article   Open access   Peer reviewed

Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus

Yoshihide Sunada, Suzanne M Bernier, Christine A Kozak, Yoshihiko Yamada and Kevin P Campbell
The Journal of biological chemistry, Vol.269(19), pp.13729-13732
1994
DOI: 10.1016/S0021-9258(17)36704-2
PMID: 8188645
url
https://doi.org/10.1016/S0021-9258(17)36704-2View
Published (Version of record) Open Access

Abstract

Merosin is the predominant laminin isoform in the basal lamina of striated muscle and peripheral nerve, and consists of M, B1 or S, and B2 chains. Here we have demonstrated that merosin is a native ligand for alpha-dystroglycan, an extracellular component of the dystrophin-glycoprotein complex. We have also mapped the mouse M chain gene, Lamm, to the same region of mouse chromosome 10 to which the dystrophia muscularis (dy) locus has been mapped. The dy mutation represents a severe neuromuscular disease resembling human muscular dystrophy. Analysis of merosin expression of dystrophic dy mice revealed a specific deficiency of merosin in skeletal muscle, cardiac muscle, and peripheral nerve. Our results indicate that merosin deficiency may be the primary defect in dy mice and suggest that a disruption of the link between alpha-dystroglycan and merosin may be involved in the pathogenesis of muscle degeneration and peripheral neuropathy in dy mice
 Neurology Biological and medical sciences Medical sciences Diseases of striated muscles. Neuromuscular diseases

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