Article
Mice harboring a R133L heterozygous mutation in LMNA exhibited ectopic lipid accumulation, aging, and mitochondrial dysfunction in adipose tissue.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Feb 2023
Qiu Ruojun, Wang Shuo, Lin Dingyi, He Yingzi, Huang Shaohan, Wu Beibei, Li Hong, Wang Min, Zheng Fenping
Abstract excerpt
The LMNA gene encodes for the nuclear envelope proteins lamin A and C (lamin A/C). A novel R133L heterozygous mutation in the LMNA gene causes atypical progeria syndrome (APS). However, the underlying mechanism remains unclear. Here, we used transgenic mice (LmnaR133L/+ mice) that expressed a heterozygous LMNA R133L mutation and 3T3-L1 cell lines with stable overexpression of LMNA R133L (by lentiviral...
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