Cloned (Comment) | Organism |
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expression in BHK cell | Homo sapiens |
Protein Variants | Comment | Organism |
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additional information | introduction of cysteine resiudes to a human CFTR variant from which all cysteines had been removed by mutagenesis and which includes a mutation in NBD1, V510A, to increase the level of protein expression in the cell membrane. Methanethiosulfonate reagents modify cysteines introduced at 14 of 16 sites studied in the juxtamembrane region of loop 3, in all cases leading to inhibition of channel function. In most cases, both the functional effects of modification and the rate of modification are similar for negatively and positively charged methanethiosulfonate reagents. Single-channel recordings indicate that, at all sites, inhibition is the result of an methanethiosulfonate reagent-induced decrease in channel open probability, in no case the conductance of open channels is altered by modification. For mutants L941C, I942C, K946C, I947C, H950C, L973C, N974C, S977C, I980C, A981C, and I982C, macroscopic currents are inhibited by both 2-sulfanoethyl methanethiosulfonate and [2-(trimethylammonium)ethyl] methanethiosulfonate. Mutant T943C, V944C, and R975C are inhibited by 2-sulfanoethyl methanethiosulfonate but not significantly affected by [2-(trimethylammonium)ethyl] methanethiosulfonate exposure. Mutants F976C and L983C, are not significantly affected by either 2-sulfanoethyl methanethiosulfonate or [2-(trimethylammonium)ethyl]methanethiosulfonate | Homo sapiens |
Organism | UniProt | Comment | Textmining |
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Homo sapiens | - |
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