The role of the UPS in cystic fibrosis

CF is an inherited autosomal recessive disease whose lethality arises from malfunction of CFTR, a single chloride (Cl(-)) ion channel protein. CF patients harbor mutations in the CFTR gene that lead to misfolding of the resulting CFTR protein, rendering it inactive and mislocalized. Hundreds of CF-r...

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Bibliografiske detaljer
Main Authors: Turnbull, Emma L, Rosser, Meredith FN, Cyr, Douglas M
Format: Artigo
Sprog:Inglês
Udgivet: BioMed Central 2007
Fag:
Review
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2106362/
https://ncbi.nlm.nih.gov/pubmed/18047735
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2091-8-S1-S11
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