Article
Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX.
The Journal of membrane biology - 1 Jul 1999
Schultz B D, Frizzell R A, Bridges R J
Abstract excerpt
Nucleotide-dependent gating of DeltaF508-CFTR was evaluated in membrane patches excised from HEK 293 and mouse L-cells and compared to observations on wt-CFTR channels recorded in the same expression systems. DeltaF508-CFTR exhibited PKA activated, ATP-dependent channel gating. When compared to wt-CFTR, the Km for ATP was increased by ninefold (260 micron vs. 28 micron) and maximal open probability (Po) was...
Topics
- 1-Methyl-3-isobutylxanthine
- Adenosine Triphosphate
- Animals
- Cell Line
- Chloride Channels
- Cystic Fibrosis Transmembrane Conductance Regulator
- Electric Conductivity
- Humans
- Ion Channel Gating
- Mutation
- Nucleotides
