Article
Progerin sequestration of PCNA promotes replication fork collapse and mislocalization of XPA in laminopathy-related progeroid syndromes.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Sept 2017
Hilton Benjamin A, Liu Ji, Cartwright Brian M, Liu Yiyong, Breitman Maya, Wang Youjie, Jones Rowdy, Tang Hui, Rusinol Antonio, Musich Phillip R, Zou Yue
Abstract excerpt
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that is caused by a point mutation in the LMNA gene, resulting in production of a truncated farnesylated-prelamin A protein (progerin). We previously reported that XPA mislocalized to the progerin-induced DNA double-strand break (DSB) sites, blocking DSB repair, which led to DSB accumulation, DNA damage responses, and early replication arrest...
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