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Literature summary for 5.6.1.6 extracted from

  • Muallem, D.; Vergani, P.
    Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator (2009), Philos. Trans. R. Soc. Lond. B Biol. Sci., 364, 247-255.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 5.6.1.6

Cloned(Commentary)

Cloned (Comment) Organism
the four domains comprising CFTR are encoded by a single gene comprising an N-terminal TMD1 and NBD1 and a C-terminal TMD2 and NBD2 Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
membrane
-
 Homo sapiens 16020
-

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O + closed Cl- channel Homo sapiens CFTR shares a common molecular mechanism with other members of the ATP-binding cassette superfamily. CFTR gating is regulated by protein kinase A-mediated phosphorylation and by interaction with ATP ADP + phosphate + open Cl- channel
-
 ?
additional information Homo sapiens a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator results in abnormal electrolyte permeability in various exocrine epithelia causing cystic fibrosis, one of the most common lethal autosomal recessive disorders in Caucasian populations ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
phosphoprotein CFTR gating is regulated by protein kinase A-mediated phosphorylation and by interaction with ATP Homo sapiens

Reaction

Reaction Comment Organism Reaction ID
ATP + H2O + closed Cl- channel = ADP + phosphate + open Cl- channel ATP binding, conformational changes, and ATPase and channel activity reaction mechanism, overview Homo sapiens
a

Source Tissue

Source Tissue Comment Organism Textmining
lung
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O + closed Cl- channel CFTR shares a common molecular mechanism with other members of the ATP-binding cassette superfamily. CFTR gating is regulated by protein kinase A-mediated phosphorylation and by interaction with ATP Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel ATP binding, at one or both nucleotide binding sites, occurs on closed channels and is required for channel opening. ATP binds to partial binding sites on the surface of the two nucleotide binding sites, NBDs, which then associate to form a NBD dimer, with complete composite catalytic sites now buried at the interface. After ATP binding, formation of a tight NBD1/NBD2 dimer is coupled to conformational changes in the transmembrane domains opening the diffusion pathway for anions. Hydrolysis at the consensus site allows fast channel closure, controlled by NBD dimerization, overview Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
additional information a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator results in abnormal electrolyte permeability in various exocrine epithelia causing cystic fibrosis, one of the most common lethal autosomal recessive disorders in Caucasian populations Homo sapiens ?
-
 ?
additional information an ATPase cycle involving formation and dissociation of tight nucleotide binding dimers drives the cyclical conformational changes in the transmembrane domains resulting in uphill allocrite transport, via ATP binding, tight NBD dimerization, hydrolysis, loss of the gamma-phosphate, and opening of the NBD-NBD interface Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
More the four domains comprising CFTR are encoded by a single gene comprising an N-terminal TMD1 and NBD1 and a C-terminal TMD2 and NBD2. In the head subdomain, conserved motifs include the Walker A and B motifs involved in nucleotide coordination and hydrolysis, while the conserved ABC signature sequence, LSGGQXXR, is in the tail subdomain Homo sapiens

Synonyms

Synonyms Comment Organism
CFTR
-
 Homo sapiens
cystic fibrosis transmembrane conductance regulator
-
 Homo sapiens
More CFTR belongs to the ATP-binding cassette superfamily Homo sapiens