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
Autores principales: Spear, Eric D., Hsu, Erh-Ting, Nie, Laiyin, Carpenter, Elisabeth P., Hrycyna, Christine A., Michaelis, Susan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2018
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078402/
https://www.ncbi.nlm.nih.gov/pubmed/29794150
http://dx.doi.org/10.1242/dmm.033670

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078402/
https://www.ncbi.nlm.nih.gov/pubmed/29794150
http://dx.doi.org/10.1242/dmm.033670

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