Article
Myopathic lamin mutations cause reductive stress and activate the nrf2/keap-1 pathway.
PLoS genetics - 1 May 2015
Dialynas George, Shrestha Om K, Ponce Jessica M, Zwerger Monika, Thiemann Dylan A, Young Grant H, Moore Steven A, Yu Liping, Lammerding Jan, Wallrath Lori L
Abstract excerpt
Mutations in the human LMNA gene cause muscular dystrophy by mechanisms that are incompletely understood. The LMNA gene encodes A-type lamins, intermediate filaments that form a network underlying the inner nuclear membrane, providing structural support for the nucleus and organizing the genome. To better understand the pathogenesis caused by mutant lamins, we performed a structural and functional analysis on...
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