Article
Targeting RAS-converting enzyme 1 overcomes senescence and improves progeria-like phenotypes of ZMPSTE24 deficiency.
Aging cell - 1 Aug 2020
Yao Haidong, Chen Xue, Kashif Muhammad, Wang Ting, Ibrahim Mohamed X, Tüksammel Elin, Revêchon Gwladys, Eriksson Maria, Wiel Clotilde, Bergo Martin O
Abstract excerpt
Several progeroid disorders are caused by deficiency in the endoprotease ZMPSTE24 which leads to accumulation of prelamin A at the nuclear envelope. ZMPSTE24 cleaves prelamin A twice: at the third carboxyl-terminal amino acid following farnesylation of a -CSIM motif; and 15 residues upstream to produce mature lamin A. The carboxyl-terminal cleavage can also be performed by RAS-converting enzyme 1 (RCE1) but...
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