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Title: Rescue of functional DeltaF508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells
Authors: Owsianik, Grzegorz ×
Cao, Lishuang
Nilius, Bernd #
Issue Date: Nov-2003
Series Title: FEBS Letters vol:554 issue:1-2 pages:173-8
Abstract: The most frequent mutant variant of the cystic fibrosis transmembrane conductance regulator (CFTR), DeltaF508-CFTR, is misprocessed and subsequently degraded in the endoplasmic reticulum. Using the patch-clamp technique, we showed that co-expressions of DeltaF508-CFTR with the N-terminal CFTR truncates containing bi-arginine (RXR) retention/retrieval motifs result in a functional rescue of the DeltaF508-CFTR mutant channel in COS-1 cells. This DeltaF508-CFTR rescue process was strongly impaired when truncated CFTR constructs possessed either the DeltaF508 mutation or arginine-to-lysine mutations in RXRs. In conclusions, our data demonstrated that expression of truncated CFTR constructs could be a novel promising approach to improve maturation of DeltaF508-CFTR channels.
ISSN: 0014-5793
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory of Ion Channel Research (VIB-KU Leuven Center for Brain & Disease Research)
Department of Cellular and Molecular Medicine - miscellaneous
× corresponding author
# (joint) last author

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