B6.WK-Lama2dy-2J/J (Dy2J)


Mutation in laminin alpha2 gene results in progressive weakness and paralysis starting at around 14 weeks of age. The hindlimbs are affected first, and later the axial and forelimb musculature. Death usually occurs before 24 weeks of age. Skeletal muscle shows degenerative changes with proliferation of sarcolemmal nuclei, an increased amount of interstitial tissue, and size variation among individual muscle fibers. C57BL/6 mice are the wild-type controls for this model. We have systematically phenotyped this model at multiple age groups.

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Sunada, Y., Bernier, S. M., Utani, A., Yamada, Y., & Campbell, K. P. (1995). Identification of a novel mutant transcript of laminin alpha 2 chain gene responsible for muscular dystrophy and dysmyelination in dy2J mice. Hum Mol Genet, 4(6), 1055-1061.

Miciak, J. J., Warsing, L. C., Tibbs, M. E., Jasper, J. R., Jampel, S. B., Malik, F. I., . . . Wagner, K. R. (2013). Fast skeletal muscle troponin activator in the dy2J muscular dystrophy model. Muscle Nerve, 48(2), 279-285.