Article
Defective prelamin A processing promotes unconventional necroptosis driven by nuclear RIPK1.
Nature cell biology - 1 Apr 2024
Yang Yuanxin, Zhang Jian, Lv Mingming, Cui Na, Shan Bing, Sun Qi, Yan Lingjie, Zhang Mengmeng, Zou Chengyu, Yuan Junying, Xu Daichao
Abstract excerpt
Defects in the prelamin A processing enzyme caused by loss-of-function mutations in the ZMPSTE24 gene are responsible for a spectrum of progeroid disorders characterized by the accumulation of farnesylated prelamin A. Here we report that defective prelamin A processing triggers nuclear RIPK1-dependent signalling that leads to necroptosis and inflammation. We show that accumulated prelamin A recruits RIPK1 to the...
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