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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Cormet-boyaka, Estelle Jablonsky, Michael Naren, Anjaparavanda P. Jackson, Patricia L. Kirk, Kevin L. Muccio, Donald D. |
| Description | Author Affiliation: Cormet-Boyaka E ( Department of Physiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA.); |
| Abstract | Most cases of cystic fibrosis (CF) are caused by mutations that block the biosynthetic maturation of the CF gene product, the CF transmembrane conductance regulator (CFTR) chloride channel. CFTR-processing mutants fail to escape the endoplasmic reticulum and are rapidly degraded. Current efforts to induce the maturation of CFTR mutants target components of the biosynthetic pathway (e.g., chaperones) rather than CFTR per se. Such methods are inherently nonspecific. Here we show that the most common CF-causing mutant (DeltaF508-CFTR) can form mature, functional chloride channels that reach the cell surface when coexpressed with several other CFTR-processing mutants or with amino fragments of the wild-type CFTR protein. This transcomplementation effect required a specific match between the region flanking the disease-causing mutation and the complementing fragment; e.g., amino fragments complemented DeltaF508-CFTR but not H1085R (a carboxy-processing mutant), whereas a carboxy fragment complemented H1085R but not DeltaF508-CFTR. Transcomplementing fragments did not affect CFTR interactions with Hsc70, a chaperone previously implicated in CFTR biosynthesis. Instead, they may promote CFTR maturation by blocking nonproductive interactions between domains within the same or neighboring CFTR polypeptides that prevent normal processing. These findings indicate that it may be possible to develop CF therapies (e.g., mini-cDNA constructs for gene therapy) that are tailored to specific disease-causing mutants of CFTR. |
| ISSN | 00278424 |
| e-ISSN | 10916490 |
| Journal | Proceedings of the National Academy of Sciences of the United States of America |
| Issue Number | 21 |
| Volume Number | 101 |
| Language | English |
| Publisher | National Academy of Sciences |
| Publisher Date | 2004-05-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Cystic Fibrosis Transmembrane Conductance Regulator Genetics Metabolism Cystic Fibrosis Mutation Protein Processing, Post-Translational Animals COS Cells Cell Line Genetic Complementation Test Glycosylation Peptide Fragments Protein Binding Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Multidisciplinary |
| Content Type | Text |
| Resource Type | Article |
| Subject | Multidisciplinary |
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