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Laminin α2 chain‐null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)‐deficient congenital muscular dystrophy
Author(s) -
Miyagoe Yuko,
Hanaoka Kazunori,
aka Ikuya,
Hayasaka Michiko,
Nabeshima Yoko,
Arahata Kiichi,
Nabeshima Yo-ichi,
Takeda Shin'ichi
Publication year - 1997
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(97)01007-7
Subject(s) - laminin , congenital muscular dystrophy , muscular dystrophy , dna laddering , mutant , biology , microbiology and biotechnology , gene , pathology , apoptosis , endocrinology , extracellular matrix , programmed cell death , genetics , medicine , dna fragmentation
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.