Meckel-Gruber Syndrome Protein MKS3 Is Required for Endoplasmic Reticulum-associated Degradation of Surfactant Protein C

Autosomal dominant mutations in the SFTPC gene are associated with idiopathic pulmonary fibrosis, a progressive lethal interstitial lung disease. Mutations that cause misfolding of the encoded proprotein surfactant protein C (SP-C) trigger endoplasmic reticulum (ER)-associated degradation, a pathway...

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Hlavní autoři: Wang, Mei, Bridges, James P., Na, Cheng-Lun, Xu, Yan, Weaver, Timothy E.
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Biochemistry and Molecular Biology 2009
Témata:
Protein Synthesis, Post-Translational Modification, and Degradation
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC2785181/
https://ncbi.nlm.nih.gov/pubmed/19815549
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M109.034371
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