Attempts of facilitated trafficking ΔF508-CFTR to the plasma membrane

Shityakov, S. W., Micaroni, M., Mironov, A .A. and Luini, A. (2007). Attempts of facilitated trafficking ΔF508-CFTR to the plasma membrane. In: Journal of Cystic Fibrosis. 30th European Cystic Fibrosis Conference, Belek, Turkey, (S7-S7). 13-16 June 2007. doi:10.1016/S1569-1993(07)60024-1

Author Shityakov, S. W.
Micaroni, M.
Mironov, A .A.
Luini, A.
Title of paper Attempts of facilitated trafficking ΔF508-CFTR to the plasma membrane
Conference name 30th European Cystic Fibrosis Conference
Conference location Belek, Turkey
Conference dates 13-16 June 2007
Proceedings title Journal of Cystic Fibrosis   Check publisher's open access policy
Place of Publication London, UK
Publisher Elsevier B.V.
Publication Year 2007
Sub-type Poster
DOI 10.1016/S1569-1993(07)60024-1
ISSN 1569-1993
Volume 6
Issue Supplement 1
Start page S7
End page S7
Total pages 1
Language eng
Abstract/Summary Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations of the Cystic Fibrosis transmembrane conductance regulator protein (CFTR), a cAMPregulated chloride channel. One of the most common mutation of CFTR is the deletion of phenylalanine in 508 position (DF508-CFTR). This mutation induces small conformational change hence CFTR trafficking is no more effective due to its rapid degradation by means of chaperon machinery. In CF airways, abnormal epithelial ion transport mainly initiates mucus stasis resulting in infections. In this study we tried to (1) understand intracellular trafficking of CFTR and DF508-CFTR, (2) facilitate transport of DF508-CFTR by means of relieving it from degradation and (3) create assay for the robotized high-throughput drugs screening. Using broad spectrum of methods from recombinant DNA and immunocitochemistry to electron microscopy, we identified main ethiologic mechanisms of cystic fibrosis. Our data demonstrate what we believe is possible to find a small molecule (adaptors and potentiators) for facilitation of DF508-CFTR trafficking to the plasma membrane. Supported by: TeleThon Grant for Cystic Fibrosis Research 2006.
Subjects 1103 Clinical Sciences
Q-Index Code EX
Q-Index Status Provisional Code
Institutional Status Unknown
Additional Notes Abstract number: 29

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Created: Fri, 05 Mar 2010, 11:30:38 EST by Laura McTaggart on behalf of Institute for Molecular Bioscience