KEGG   ENZYME: 3.6.3.49Help
Entry
EC 3.6.3.49                 Enzyme                                 
Name
channel-conductance-controlling ATPase
Class
Hydrolases;
Acting on acid anhydrides;
Acting on acid anhydrides to catalyse transmembrane movement of substances
BRITE hierarchy
Sysname
ATP phosphohydrolase (channel-conductance-controlling)
Reaction(IUBMB)
ATP + H2O = ADP + phosphate [RN:R00086]
Reaction(KEGG)
Substrate
ATP [CPD:C00002];
H2O [CPD:C00001]
Product
ADP [CPD:C00008];
phosphate [CPD:C00009]
Comment
ABC-type (ATP-binding cassette-type) ATPase, characterized by the presence of two similar ATP-binding domains. Does not undergo phosphorylation during the transport process. An animal enzyme that is active in forming a chloride channel, the absence of which brings about cystic fibrosis. It is also involved in the functioning of other transmembrane channels.
Orthology
K05031  
cystic fibrosis transmembrane conductance regulator
Genes
HSA: 
1080(CFTR)
PTR: 
463674(CFTR)
PPS: 
100989405(CFTR)
GGO: 
101132066(CFTR)
PON: 
100137035(CFTR)
MCC: 
574346(CFTR)
MMU: 
12638(Cftr)
RNO: 
24255(Cftr)
CFA: 
492302(CFTR)
AML: 
FCA: 
492308(CFTR)
BTA: 
281067(CFTR)
SSC: 
403154(CFTR)
ECB: 
100071259(CFTR)
MDO: 
100014119
SHR: 
100919086(CFTR)
OAA: 
100078764(CFTR)
GGA: 
100049619(CFTR)
MGP: 
100545354
TGU: 
100222243(CFTR)
ACS: 
100559531
XTR: 
100486085
DRE: 
559080(cftr)
TRU: 
492310(cftr)
OLA: 
101161380
 » show all
Taxonomy
Reference
1  [PMID:9147660]
  Authors
Chen M, Zhang JT.
  Title
Membrane insertion, processing, and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes.
  Journal
Mol. Membr. Biol. 13 (1996) 33-40.
  Organism
Homo sapiens [GN:hsa], Oryctolagus cuniculus
Reference
2  [PMID:9013845]
  Authors
Tusnady GE, Bakos E, Varadi A, Sarkadi B.
  Title
Membrane topology distinguishes a subfamily of the ATP-binding cassette (ABC) transporters.
  Journal
FEBS. Lett. 402 (1997) 1-3.
  Organism
Homo sapiens [GN:hsa]
Reference
3  [PMID:9922375]
  Authors
Sheppard DN, Welsh MJ.
  Title
Structure and function of the CFTR chloride channel.
  Journal
Physiol. Rev. 79 (1999) S23-45.
  Organism
human [GN:hsa]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
DBGET integrated database retrieval system