Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI).

Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Research Abstract Details 

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Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Abstract Text:

Ying Yu,Oleksandr Platoshyn,Olga Safrina,Igor Tsigelny,Jason X-J Yuan,Steven H Keller,

BACKGROUND INFORMATION: Cystic fibrosis results from mutations in the ABC transporter CFTR (cystic fibrosis transmembrane conductance regulator), which functions as a cAMP-regulated anion channel. The most prevalent mutation in CFTR, the Phe(508) deletion, results in the generation of a trafficking and functionally deficient channel. The cellular machineries involved in modulating CFTR trafficking and function have not been fully characterized. In the present study, we identified a role for the COPI (coatomer protein I) cellular trafficking machinery in the development of the CFTR polypeptide into a functional chloride channel. To examine the role of COPI in CFTR biosynthesis, we employed the cell line ldlF, which harbours a temperature-sensitive mutation in epsilon-COP, a COPI subunit, to inhibit COPI function and then determined whether the CFTR polypeptide produced from the transfected gene developed into a cAMP-regulated chloride channel. RESULTS: When COPI was inactivated in the ldlF cells by an elevated temperature pulse (39 degrees C), the CFTR polypeptide was detected on the cell surface by immunofluorescence microscopy and cell-surface biotinylation. Therefore, CFTR proceeded upstream in the secretory pathway in the absence of COPI function, a result demonstrated previously by others. In contrast, electrophysiological measurements indicated an absence of cAMP-stimulated chloride efflux, suggesting that channel function was impaired. In comparison, expression of CFTR at the same elevated temperature (39 degrees C) in an epsilon-COP-rescued cell line [ldlF(ldlF)], which has an introduced wild-type epsilon-COP gene in addition to the mutant epsilon-COP gene, showed restoration of cAMP-stimulated channel activity, confirming the requirement of COPI for channel function. CONCLUSIONS: These results therefore suggest that generation of the folded-functional conformation of CFTR requires COPI.

Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Publishing Authors By Initials

Y Yu,O Platoshyn,O Safrina,I Tsigelny,JX Yuan,SH Keller,

For similar carbohydrates: polysaccharides: oligosaccharides research abstracts see: carbohydrates: polysaccharides: oligosaccharides research

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Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Biology of the cell / under the auspices of the Eu

VOLUME: 99

Page Numbers: 433-44

Journal Abbreviation: Biol. Cell

ISSN: 1768-322X

DAY: 3

MONTH: Aug

YEAR: 2007

Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 8108529

Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Keywords Mesh Terms:

KEYWORDS: Oligosaccharides

MESH TERMS: metabolism

Chemical & Substance for Abstract: Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI). Information

Substance Name: Forskolin

Registry Number: 66428-89-5

Grant and Affiliation Information for Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI).

AFFILIATION: Department of Medicine, University of California, San Diego, La Jolla, CA 92093-0725, USA.

Country: England

AGENCY: United States NHLBI

GRANT: HL66012

ACRONYM: HL

MEDLINETA: Biol Cell

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