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

• Main Authors: Eric D. Spear (Author), Erh-Ting Hsu (Author), Laiyin Nie (Author), Elisabeth P. Carpenter (Author), Christine A. Hrycyna (Author), Susan Michaelis (Author)
• Format: Book
• Published: The Company of Biologists, 2018-07-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...