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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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Bibliografiske detaljer
Udgivet i:Dis Model Mech
Main Authors: Spear, Eric D., Hsu, Erh-Ting, Nie, Laiyin, Carpenter, Elisabeth P., Hrycyna, Christine A., Michaelis, Susan
Format: Artigo
Sprog:Inglês
Udgivet: The Company of Biologists Ltd 2018
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6078402/
https://ncbi.nlm.nih.gov/pubmed/29794150
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.033670
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