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Literature summary for 5.6.1.6 extracted from

  • Sheppard, D.N.; Welsh, M.J.
    Structure and function of the CFTR chloride channel (1999), Physiol. Rev., 79, S23-45.
    View publication on PubMed
Search for more literature for 5.6.1.6

Activating Compound

Activating Compound Comment Organism Structure
BeF3 amount of Cl- currents is less than 30% that of the wild-type CFTR Homo sapiens
diphosphate amount of Cl- currents is less than 30% that of the wild-type CFTR Homo sapiens
additional information activation of the cAMP-dependent protein kinase causes the phosphorylation of multiple serine residues within the R domain of CFTR. Once the R domain is phosphorylated, channel gating is regulated by a cycle of ATP hydrolysis at the nucleotide-binding domains. Finally, protein phosphatases dephosphorylate the R domain and return the channel to its quiescent state Homo sapiens
VO43- prolongs the duration of the burst of channel activity Homo sapiens

Protein Variants

Protein Variants Comment Organism
K335E conversion from a low I- permeability pore to a high I- permeability pore Homo sapiens
K95D conversion from a low I- permeability pore to a high I- permeability pore Homo sapiens
R334W amount of Cl- currents is less than 30% that of the wild-type CFTR Homo sapiens
R347P amount of Cl- currents is less than 30% that of the wild-type CFTR Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
membrane
-
 Xenopus sp. 16020
-
membrane apical membrane of epithelia Homo sapiens 16020
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O + closed Cl- channel Homo sapiens mediates transepithelial salt and lipid movement in the apical membrane of epithelia ADP + phosphate + open Cl- channel
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-
shark
-
-
-
Xenopus sp.
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
epithelium
-
 Xenopus sp.
-
epithelium apical membrane of epithelia Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O + closed Cl- channel chloride channel Mus musculus ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel chloride channel Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel chloride channel Xenopus sp. ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel chloride channel shark ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel CFTR is a multiion-pore Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel dysfunction of CFTR causes the genetic disease cystic fibrosis Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel transport of Cl-, I-, and with lower efficiency Br- and F- Homo sapiens ADP + phosphate + open Cl- channel
-
 ?
ATP + H2O + closed Cl- channel mediates transepithelial salt and lipid movement in the apical membrane of epithelia Homo sapiens ADP + phosphate + open Cl- channel
-
 ?

Subunits

Subunits Comment Organism
More enzyme of the ABC transporter family Homo sapiens
More CFTR is composed of five domains: two membrane-spanning domains which form the channel pore, two nucleotide-binding domains and a regulatory R domain. Phosphorylation of the R domain determines channel activity, ATP hydrolysis by the nucleotide-binding domains controls gating Homo sapiens