Article
Can correcting the ΔF508-CFTR proteostasis-defect rescue CF lung disease?
Current molecular medicine - 1 Aug 2012
Valle C W, Vij N
Abstract excerpt
Protein homeostasis (proteostasis) generates and maintains individual proteins in their folded and functional-competent states. The components of the cellular proteostasis machinery also dictate the functional lifetime of a protein by constantly regulating its conformation, concentration and subcellular location. The autosomal recessive disease cystic fibrosis (CF) is caused by a proteostasis-defect in CF...
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