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Literature summary extracted from

  • Fatehi, M.; Linsdell, P.
    State-dependent access of anions to the cystic fibrosis transmembrane conductance regulator chloride channel pore (2008), J. Biol. Chem., 283, 6102-6109.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
5.6.1.6 cAMP stimulates the ion channel function Homo sapiens
5.6.1.6 diphosphate activates the enzyme ion channel Homo sapiens
5.6.1.6 KCN without cAMP stimulation, KCN treatment increases CFTR Cl- conductance by 1.95fold, whereas after cAMP stimulation KCN treatment increases conductance by 13.7fold Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
5.6.1.6 F337C the cysteine introduced at a position in the pore-lining TM6 region of CFTR is accessible to extracellular methanesulfonate reagents, the mutant becomes significantly more inwardly rectified in the presence of sodium (2-sulfonatoethyl)methanesulfonate Homo sapiens
5.6.1.6 K335C the cysteine introduced at a position in the pore-lining TM6 region of CFTR is accessible to extracellular methanesulfonate reagents Homo sapiens
5.6.1.6 R334C the cysteine introduced at a position in the pore-lining TM6 region of CFTR is accessible to extracellular methanesulfonate reagents Homo sapiens
5.6.1.6 S341C the cysteine introduced at a position in the pore-lining TM6 region of CFTR is accessible to extracellular methanesulfonate reagents, charge-dependent changes in I-V shape in this mutant, indicating that deposition of charge at this position also alters anion movement in the pore Homo sapiens
5.6.1.6 T338C Au(CN)2 - can modify T338C-CFTR with or without cAMP stimulation in contrast to the wild-type enzyme Homo sapiens
5.6.1.6 T338C the cysteine introduced at a position in the pore-lining TM6 region of CFTR is accessible to extracellular methanesulfonate reagents Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
5.6.1.6 Au(CN)2- inhibits the ion channel function Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
5.6.1.6 Mg2+ required Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
5.6.1.6 additional information Homo sapiens the enzyme acts as an ion channel, e.g. transporting chloride and the anionic cysteine-reactive reagents methanesulfonate sodium (2-sulfonatoethyl)methanesulfonate, organic mercurial 4-chloromercuriphenylsulfonic acid, and the permeant anion Au(CN)2-, but only in activated status, in nonactivated channels some ion selectivity mechanism exists to exclude anions yet permit cations into the channel pore from the extracellular solution. Activation of CFTR channels involves a conformational change in the pore that removes a strong selectivity against anion entry from the extracellular solution ?
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 ?

Organism

EC Number Organism UniProt Comment Textmining
5.6.1.6 Homo sapiens
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-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
5.6.1.6 additional information the enzyme acts as an ion channel, e.g. transporting chloride and the anionic cysteine-reactive reagents methanesulfonate sodium (2-sulfonatoethyl)methanesulfonate, organic mercurial 4-chloromercuriphenylsulfonic acid, and the permeant anion Au(CN)2-, but only in activated status, in nonactivated channels some ion selectivity mechanism exists to exclude anions yet permit cations into the channel pore from the extracellular solution. Activation of CFTR channels involves a conformational change in the pore that removes a strong selectivity against anion entry from the extracellular solution Homo sapiens ?
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 ?

Synonyms

EC Number Synonyms Comment Organism
5.6.1.6 cystic fibrosis transmembrane conductance regulator
-
 Homo sapiens