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Title: Phosphorylation site independent single R-domain mutations affect CFTR channel activity
Authors: Wei, L ×
Vankeerberghen, A
Cuppens, Harry
Droogmans, Guillaume
Cassiman, Jean-Jacques
Nilius, Bernd #
Issue Date: Nov-1998
Publisher: Elsevier science bv
Series Title: FEBS Letters vol:439 issue:1-2 pages:121-6
Abstract: We investigated CFTR channel activity of mature R-domain mutants showing single alterations at sites other than the predicted phosphorylation sites. All mutations were found in cystic fibrosis (CF) patients (H620Q, E822K and E826K). The macroscopic CFTR chloride conductance induced by phosphorylation was significantly enhanced in Xenopus oocytes injected with mRNA of H620Q but reduced in the E822K and E826K mutants compared to wild type CFTR. The anion permeability sequence for all three mutants was the same as that of wild type CFTR. Cell attached single channel studies in COS cells revealed that both open channel probability and/or the number of functional channels were either higher (H620Q) or lower (E822K and E826K) than in wild type CFTR. Single channel conductances were unchanged in all mutants. Our results suggest that additional sites in the R-domain other than phosphorylation sites influence gating of CFTR channels.
URI: 
ISSN: 0014-5793
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Department of Human Genetics - miscellaneous
Laboratory of Ion Channel Research
Human Mutations and Polymorphisms Section (-)
Physiology Section (-)
Department of Cellular and Molecular Medicine - miscellaneous
Forensic Biomedical Sciences
× corresponding author
# (joint) last author

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