Laminin α2 chain‐null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)‐deficient congenital muscular dystrophy
Copyright policy )Laminin α2 chain‐null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)‐deficient congenital muscular dystrophy
Using the gene targeting technique, we have generated a new mouse model of congenital muscular dystrophy (CMD), a null mutant for the laminin α2 chain. These homozygous mice, designated dy3K/dy3K , are characterized by growth retardation and severe muscular dystrophic symptoms and die by 5 weeks of age. Light microscopy revealed that muscle fiber degeneration in these mice begins no later than postnatal day 9. In degenerating muscles, considerable amounts of TUNEL positive nuclei were detected as well as DNA laddering, suggesting increased apoptotic cell death was involved in the process of muscle fiber degeneration.
- Osaka University Japan
- Kitasato University Japan
- National Center of Neurology and Psychiatry Japan
- Kitasato University Japan
Blotting, Western, Genetic Vectors, Muscle Fibers, Skeletal, Skeletal muscle, Apoptosis, DNA Fragmentation, Mice, Animals, Muscle, Skeletal, Basal lamina, Cell Nucleus, Mice, Knockout, Histocytochemistry, Myocardium, Gene targeting, Muscular Dystrophy, Animal, Muscular dystrophy, Immunohistochemistry, Blotting, Southern, Disease Models, Animal, Microscopy, Electron, Laminin α2 chain, Gene Targeting, Mutation, Laminin
Blotting, Western, Genetic Vectors, Muscle Fibers, Skeletal, Skeletal muscle, Apoptosis, DNA Fragmentation, Mice, Animals, Muscle, Skeletal, Basal lamina, Cell Nucleus, Mice, Knockout, Histocytochemistry, Myocardium, Gene targeting, Muscular Dystrophy, Animal, Muscular dystrophy, Immunohistochemistry, Blotting, Southern, Disease Models, Animal, Microscopy, Electron, Laminin α2 chain, Gene Targeting, Mutation, Laminin
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