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Diminished Self-Chaperoning Activity of the ΔF508 Mutant of CFTR Results in Protein Misfolding
The absence of a functional ATP Binding Cassette (ABC) protein called the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) from apical membranes of epithelial cells is responsible for cystic fibrosis (CF). Over 90% of CF patients carry at least one mutant allele with deletion of phenylalan...
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| Main Authors: | , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Public Library of Science
2008
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2265529/ https://ncbi.nlm.nih.gov/pubmed/18463704 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pcbi.1000008 |
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