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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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Detalles Bibliográficos
Publicado en:Dis Model Mech
Main Authors: Spear, Eric D., Hsu, Erh-Ting, Nie, Laiyin, Carpenter, Elisabeth P., Hrycyna, Christine A., Michaelis, Susan
Formato: Artigo
Idioma:Inglês
Publicado: The Company of Biologists Ltd 2018
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Acceso en liña: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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