Article
The cystic-fibrosis-associated ΔF508 mutation confers post-transcriptional destabilization on the C. elegans ABC transporter PGP-3.
Disease models & mechanisms - 1 Nov 2012
He Liping, Skirkanich Jennifer, Moronetti Lorenza, Lewis Rosemary, Lamitina Todd
Abstract excerpt
Membrane proteins make up ∼30% of the proteome. During the early stages of maturation, this class of proteins can experience localized misfolding in distinct cellular compartments, such as the cytoplasm, endoplasmic reticulum (ER) lumen and ER membrane. ER quality control (ERQC) mechanisms monitor folding and determine whether a membrane protein is appropriately folded or is misfolded and warrants degradation....
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