KEGG   ENZYME: 5.6.1.6
Entry
EC 5.6.1.6                  Enzyme                                 

Name
channel-conductance-controlling ATPase;
cystic fibrosis transmembrane conductance regulator;
CFTR (gene name)
Class
Isomerases;
Isomerases altering macromolecular conformation;
Enzymes altering polypeptide conformation or assembly
Sysname
ATP phosphohydrolase (channel-conductance-controlling)
Reaction(IUBMB)
ATP + H2O + closed Cl- channel = ADP + phosphate + open Cl- channel
Substrate
ATP [CPD:C00002];
H2O [CPD:C00001];
closed Cl- channel
Product
ADP [CPD:C00008];
phosphate [CPD:C00009];
open Cl- channel
Comment
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.
History
EC 5.6.1.6 created 2000 as EC 3.6.3.49, transferred 2018 to EC 5.6.1.6
Orthology
K05031  cystic fibrosis transmembrane conductance regulator
Genes
HSA: 1080(CFTR)
PTR: 463674(CFTR)
PPS: 100989405(CFTR)
GGO: 101132066(CFTR)
PON: 100137035(CFTR)
NLE: 100601480(CFTR)
MCC: 574346(CFTR)
MCF: 102130331(CFTR)
CSAB: 103226777(CFTR)
RRO: 104682625(CFTR)
RBB: 108526537(CFTR)
CJC: 100415251(CFTR)
SBQ: 101036459(CFTR)
MMU: 12638(Cftr)
MCAL: 110296314(Cftr)
MPAH: 110316437(Cftr)
RNO: 24255(Cftr)
MUN: 110564980(Cftr) 110564982
CGE: 100754506(Cftr)
NGI: 103737361(Cftr)
HGL: 101709472(Cftr)
CCAN: 109679300(Cftr)
OCU: 100009471(CFTR)
TUP: 102490714(CFTR)
CFA: 492302(CFTR)
VVP: 112929896(CFTR)
AML: 100478723(CFTR)
UMR: 103676590(CFTR)
UAH: 113263308(CFTR)
ORO: 101363648(CFTR)
ELK: 111152043
FCA: 492308(CFTR)
PTG: 102956215(CFTR)
PPAD: 109248378(CFTR)
AJU: 106970664(CFTR)
BTA: 281067(CFTR)
BOM: 102264773(CFTR)
BIU: 109557891(CFTR)
BBUB: 102397474(CFTR)
CHX: 102179446(CFTR)
OAS: 443347(CFTR)
SSC: 403154(CFTR)
CFR: 102505598(CFTR)
CDK: 105085325(CFTR)
BACU: 103004581(CFTR)
LVE: 103088529(CFTR)
OOR: 101278319(CFTR)
DLE: 111179250(CFTR)
PCAD: 102973776(CFTR)
ECB: 100071259(CFTR)
EPZ: 103550601(CFTR)
EAI: 106829379(CFTR)
MYB: 102257625(CFTR)
MYD: 102773631(CFTR)
MNA: 107524880(CFTR)
HAI: 109376431
DRO: 112300407(CFTR)
PALE: 102884577(CFTR)
RAY: 107519971(CFTR)
MJV: 108387496
LAV: 100662653(CFTR)
TMU: 101346801
MDO: 100014119(CFTR)
SHR: 100919086(CFTR)
PCW: 110200665(CFTR)
OAA: 100078764(CFTR)
GGA: 100049619(CFTR)
MGP: 100545354(CFTR)
CJO: 107309076(CFTR)
NMEL: 110392590(CFTR)
APLA: 101803386(CFTR)
ACYG: 106042053(CFTR)
TGU: 100222243(CFTR)
LSR: 110483509(CFTR)
SCAN: 103813684(CFTR)
GFR: 102041165(CFTR)
FAB: 101809710(CFTR)
PHI: 102114534(CFTR)
PMAJ: 107205064(CFTR)
CCAE: 111937028(CFTR)
CCW: 104698445(CFTR)
ETL: 114062358(CFTR)
FPG: 101921929(CFTR)
FCH: 102050923(CFTR)
CLV: 102089481(CFTR)
EGZ: 104133415(CFTR)
NNI: 104013586(CFTR)
ACUN: 113491166(CFTR)
PADL: 103917004(CFTR)
AAM: 106488125(CFTR)
ASN: 102369471(CFTR)
AMJ: 102563163(CFTR)
PSS: 102447829(CFTR)
CMY: 102936252(CFTR)
CPIC: 101933840(CFTR)
ACS: 100559531(cftr)
PVT: 110077041(CFTR)
PBI: 103057487(CFTR)
PMUR: 107285369(CFTR)
TSR: 106538296(CFTR)
PMUA: 114605368(CFTR)
GJA: 107114507(CFTR)
XLA: 108712361(cftr.S) 373725(cftr.L)
XTR: 100486085(cftr)
NPR: 108802076(CFTR)
DRE: 559080(cftr)
SANH: 107660943(cftr)
CCAR: 109110485(cftr) 109110487
IPU: 108264684(cftr)
PHYP: 113534512(cftr)
AMEX: 103032690(cftr)
EEE: 113573602(cftr)
TRU: 492310(cftr)
LCO: 104925520(cftr)
MZE: 101473042(cftr)
ONL: 100710776(cftr)
OLA: 101161380(cftr)
XMA: 102231389(cftr)
XCO: 114143601(cftr)
PRET: 103465638(cftr)
CVG: 107083207(cftr)
NFU: 107388351(cftr)
KMR: 108239387(cftr)
ALIM: 106518846(cftr)
AOCE: 111570180(cftr)
CSEM: 103379580(cftr)
POV: 109624104(cftr)
LCF: 108893910(cftr)
SDU: 111220518(cftr)
SLAL: 111672163(cftr)
HCQ: 109530830(cftr)
BPEC: 110155111(cftr)
MALB: 109956423(cftr)
OTW: 112252645
ELS: 105029518(cftr)
SFM: 108931616(cftr)
PKI: 111842430(cftr)
LCM: 102346483(CFTR)
CMK: 103179362(cftr)
RTP: 109925496(cftr)
 » show all
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 13:33-40 (1996)
DOI:10.3109/09687689609160572
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 402:1-3 (1997)
DOI:10.1016/S0014-5793(96)01478-0
Reference
3  [PMID:9922375]
  Authors
Sheppard DN, Welsh MJ.
  Title
Structure and function of the CFTR chloride channel.
  Journal
Physiol Rev 79:S23-45 (1999)
DOI:10.1152/physrev.1999.79.1.S23
Reference
4  [PMID:19332488]
  Authors
Hwang TC, Sheppard DN
  Title
Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation.
  Journal
J Physiol 587:2151-61 (2009)
DOI:10.1113/jphysiol.2009.171595
Other DBs
ExplorEnz - The Enzyme Database: 5.6.1.6
IUBMB Enzyme Nomenclature: 5.6.1.6
ExPASy - ENZYME nomenclature database: 5.6.1.6
BRENDA, the Enzyme Database: 5.6.1.6

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