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The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants

Most patients with CF (cystic fibrosis) express a CFTR [CF TM (transmembrane) conductance regulator] processing mutant that is not trafficked to the cell surface because it is retained in the endoplasmic reticulum due to altered packing of the TM segments. CL4 (cytoplasmic loop 4) connecting TMs 10...

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Autors principals: Loo, Tip W., Bartlett, M. Claire, Wang, Ying, Clarke, David M.
Format: Artigo
Idioma:Inglês
Publicat: Portland Press Ltd. 2006
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC1462697/
https://ncbi.nlm.nih.gov/pubmed/16417523
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20060013
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