Laminin α2 chain‐null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)‐deficient congenital muscular dystrophy

FEBS Letters

Nabeshima, Yo-ichi⁴  Nonaka, Ikuya¹  Arahata, Kiichi²  Nabeshima, Yoko⁴  Takeda, Shin'ichi⁴  Miyagoe, Yuko²  Hayasaka, Michiko³  Hanaoka, Kazunori³ 

[1] Department of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-higashi, Kodaira, Tokyo 187, Japan;Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-higashi, Kodaira, Tokyo 187, Japan;Department of Biosciences, School of Science, Kitasato University, 1-15-1 Kitasato, Sagamihara, Kanagawa 228, Japan;Department of Molecular Genetics, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-higashi, Kodaira, Tokyo 187, Japan

关键词: Laminin α2 chain;    Gene targeting;    Skeletal muscle;    Basal lamina;    Apoptosis;    Muscular dystrophy;   

DOI  :  10.1016/S0014-5793(97)01007-7

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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 dy^3K/dy^3K , 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.

【 授权许可】

Unknown