ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability

ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability

The human zinc metalloprotease ZMPSTE24 is an integral membrane protein crucial for the final step in the biogenesis of the nuclear scaffold protein lamin A, encoded by LMNA. After farnesylation and carboxyl methylation of its C-terminal CAAX motif, the lamin A precursor (prelamin A) undergoes prote...

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Bibliographic Details
Main Authors: Eric D. Spear, Erh-Ting Hsu, Laiyin Nie, Elisabeth P. Carpenter, Christine A. Hrycyna, Susan Michaelis
Format: Article
Language:English
Published: The Company of Biologists 2018-07-01
Series:Disease Models & Mechanisms
Subjects:
Lamin A processing
Saccharomyces cerevisiae
Ubiquitin-proteasome system
Progeria disease
Online Access:http://dmm.biologists.org/content/11/7/dmm033670

Internet

http://dmm.biologists.org/content/11/7/dmm033670

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