CATH Classification

Domain Context

CATH Clusters

Superfamily P-loop containing nucleotide triphosphate hydrolases
Functional Family

Enzyme Information

5.6.1.6
Channel-conductance-controlling ATPase.
based on mapping to UniProt P13569
ATP + H(2)O + closed Cl(-) channel = ADP + phosphate + open Cl(-) channel.
-!- ABC-type (ATP-binding cassette-type) ATPase, characterized by the presence of two similar ATP-binding domains. -!- The enzyme is found in animals, and in humans its absence brings about cystic fibrosis. -!- Unlike most of the ABC transporters, chloride pumping is not directly coupled to ATP hydrolysis. -!- Instead, the passive flow of anions through the channel is gated by cycles of ATP binding and hydrolysis by the ATP-binding domains. -!- The enzyme is also involved in the functioning of other transmembrane channels. -!- Formerly EC 3.6.3.49.

UniProtKB Entries (1)

P13569
CFTR_HUMAN
Homo sapiens
Cystic fibrosis transmembrane conductance regulator

PDB Structure

PDB 5TGK
External Links
Method X-RAY DIFFRACTION
Organism
Primary Citation
Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator.
Wang, C., Aleksandrov, A.A., Yang, Z., Forouhar, F., Proctor, E.A., Kota, P., An, J., Kaplan, A., Khazanov, N., Boel, G., Stockwell, B.R., Senderowitz, H., Dokholyan, N.V., Riordan, J.R., Brouillette, C.G., Hunt, J.F.
J. Biol. Chem.
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