Article
Lama1 upregulation prolongs the lifespan of the dyH/dyH mouse model of LAMA2-related congenital muscular dystrophy.
Journal of genetics and genomics = Yi chuan xue bao - 1 Oct 2024
Liu Yidan, Tan Dandan, Ma Kaiyue, Luo Huaxia, Mao Jingping, Luo Jihang, Shen Qiang, Xu Luzheng, Yang Shiqi, Ge Lin, Guo Yuxuan, Zhang Hong, Xiong Hui
Abstract excerpt
LAMA2-related congenital muscular dystrophy (LAMA2-CMD), characterized by laminin-α2 deficiency, is debilitating and ultimately fatal. To date, no effective therapy has been clinically available. Laminin-α1, which shares significant similarities with laminin-α2, has been proven as a viable compensatory modifier. To evaluate its clinical applicability, we establish a Lama2 exon-3-deletion mouse model (dyH/dyH)....
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