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
HSA1080(CFTR)
PTR463674(CFTR)
PPS100989405(CFTR)
GGO101132066(CFTR)
PON100137035(CFTR)
NLE100601480(CFTR)
HMH116810950(CFTR)
MCC574346(CFTR)
MCF102130331(CFTR)
MTHB126951193
MNI105475504(CFTR)
CSAB103226777(CFTR)
CATY105598482(CFTR)
PANU100126675(CFTR)
TGE112620581(CFTR)
MLEU105533272(CFTR)
RRO104682625(CFTR)
RBB108526537(CFTR)
TFN117072767(CFTR)
PTEH111553406(CFTR)
CANG105508520(CFTR)
CJC100415251(CFTR)
SBQ101036459(CFTR)
CIMI108308994(CFTR)
CSYR103264109(CFTR)
MMUR105869370(CFTR)
LCAT123646968(CFTR)
PCOQ105817115(CFTR)
OGA100961312(CFTR)
MMU12638(Cftr)
MCAL110296314(Cftr)
MPAH110316437(Cftr)
RNO24255(Cftr)
MCOC116098609(Cftr)
ANU117720697(Cftr)
MUN110564980(Cftr)
CGE100754506(Cftr)
MAUA101831516(Cftr)
PROB127215341(Cftr)
PLEU114694716(Cftr)
MORG121463441(Cftr)
MFOT126503972
AAMP119807517(Cftr)
NGI103737361(Cftr)
HGL101709472(Cftr)
CPOC100719898(Cftr)
CCAN109679300(Cftr)
DORD105988326(Cftr)
DSP122121761(Cftr)
PLOP125345484(Cftr)
NCAR124991796
MMMA107144405(Cftr)
OCU100009471(CFTR)
OPI101535310(CFTR)
TUP102490714(CFTR)
GVR103597253(CFTR)
CFA492302(CFTR)
CLUD112670805(CFTR)
VVP112929896(CFTR)
VLG121475063(CFTR)
NPO129502846(CFTR)
AML100478723(CFTR)
UMR103676590(CFTR)
UAH113263308(CFTR)
UAR123789030(CFTR)
ELK111152043
LLV125081083
MPUF101672484(CFTR)
MNP132015768(CFTR)
MLK131829662(CFTR)
NVS122905012(CFTR)
ORO101363648(CFTR)
EJU114210604(CFTR)
ZCA113911768(CFTR)
MLX118020550(CFTR)
NSU110588952(CFTR)
LWW102736525(CFTR)
FCA492308(CFTR)
PYU121028439(CFTR)
PCOO112866723(CFTR)
PBG122488303(CFTR)
LRUF124524870
PTG102956215(CFTR)
PPAD109248378(CFTR)
PUC125930080
AJU106970664
HHV120224616
BTA281067(CFTR)
BOM102264773(CFTR)
BIU109557891(CFTR)
BBUB102397474(CFTR)
BBIS104981947(CFTR)
CHX102179446(CFTR)
OAS443347(CFTR)
BTAX128046774(CFTR)
ODA120877516(CFTR)
CCAD122438337(CFTR)
MBEZ129552417(CFTR)
SSC403154(CFTR)
CFR102505598(CFTR)
CBAI105063144(CFTR)
CDK105085325(CFTR)
VPC102527896(CFTR)
BACU103004581(CFTR)
LVE103088529(CFTR)
OOR101278319(CFTR)
DLE111179250(CFTR)
PCAD102973776(CFTR)
PSIU116759844(CFTR)
NASI112408186(CFTR)
ECB100071259(CFTR)
EPZ103550601(CFTR)
EAI106829379(CFTR)
MYB102257625(CFTR)
MYD102773631(CFTR)
MMYO118666477(CFTR)
MLF102419125(CFTR)
MNA107524880(CFTR)
PKL118711824(CFTR)
EFUS103295955(CFTR)
HAI109376431
DRO112300407(CFTR)
SHON118999581(CFTR)
AJM119035603(CFTR)
PDIC114507755(CFTR)
PHAS123824765(CFTR)
MMF118619948(CFTR)
RFQ117018340(CFTR)
PPAM129063035(CFTR)
PALE102884577(CFTR)
PGIG120601125(CFTR)
PVP105308468(CFTR)
RAY107519971(CFTR)
MJV108387496(CFTR)
TOD119250439(CFTR)
SARA101556770(CFTR)
LAV100662653(CFTR)
TMU101346801
ETF101657112(CFTR)
DNM101433838(CFTR)
MDO100014119(CFTR)
GAS123249455(CFTR)
SHR100919086(CFTR)
AFZ127538624
PCW110200665(CFTR)
OAA100078764(CFTR)
GGA100049619(CFTR)
PCOC116243921(CFTR)
MGP100545354(CFTR)
CJO107309076(CFTR)
TPAI128073470(CFTR)
LMUT125694041(CFTR)
NMEL110392590(CFTR)
APLA101803386(CFTR)
ACYG106042053(CFTR)
CATA118246773(CFTR)
AFUL116501959(CFTR)
TGU100222243(CFTR)
LSR110483509(CFTR)
SCAN103813684(CFTR)
PMOA120510781(CFTR)
OTC121344821(CFTR)
PRUF121355836(CFTR)
GFR102041165(CFTR)
FAB101809710(CFTR)
OMA130248254(CFTR)
PHI102114534(CFTR)
PMAJ107205064(CFTR)
CCAE111937028(CFTR)
CCW104698445(CFTR)
CBRC103614852(CFTR)
ETL114062358(CFTR)
ZAB102064789(CFTR)
ACHL103799685(CFTR)
SVG106862907(CFTR)
MMEA130589221(CFTR)
HRT120751779(CFTR)
FPG101921929(CFTR)
FCH102050923(CFTR)
CLV102089481(CFTR)
EGZ104133415(CFTR)
NNI104013586(CFTR)
PCRI104029724(CFTR)
PLET104627445(CFTR)
EHS104506564(CFTR)
PCAO104049962(CFTR)
ACUN113491166(CFTR)
TALA104355842(CFTR)
PADL103917004(CFTR)
AFOR103905223(CFTR)
ACHC115342266(CFTR)
HALD104317503
HLE104835814(CFTR)
AGEN126044249
GCL127022852
CCRI104161185(CFTR)
CSTI104559106(CFTR)
CMAC104473521(CFTR)
MUI104537232(CFTR)
BREG104636612(CFTR)
FGA104081922
GSTE104256497(CFTR)
LDI104351295(CFTR)
MNB103778346(CFTR)
OHA104337503(CFTR)
NNT104409589(CFTR)
SHAB115604828(CFTR)
DPUB104309179(CFTR)
PGUU104472123(CFTR)
ACAR104522661(CFTR)
CPEA104394692(CFTR)
AVIT104272747
CVF104285252(CFTR)
RTD128904165(CFTR)
CUCA104064321(CFTR)
TEO104377114(CFTR)
BRHI104494230(CFTR)
AAM106488125(CFTR)
AROW112965500(CFTR)
NPD112942325(CFTR)
TGT104580765(CFTR)
DNE112981586(CFTR)
SCAM104153576(CFTR)
ASN102369471(CFTR)
AMJ102563163(CFTR)
CPOO109315988(CFTR)
GGN109296354(CFTR)
PSS102447829(CFTR)
CMY102936252(CFTR)
CCAY125636148(CFTR)
DCC119850071(CFTR)
CPIC101933840(CFTR)
TST117870523(CFTR)
CABI116838613(CFTR)
MRV120388529(CFTR)
ACS100559531(cftr)
ASAO132776261(CFTR)
PVT110077041(CFTR)
SUND121932080(CFTR)
PBI103057487(CFTR)
PMUR107285369(CFTR)
CTIG120308230(CFTR)
TSR106538296(CFTR)
PGUT117673597(CFTR)
APRI131202459(CFTR)
PTEX113444417(CFTR)
NSS113413065(CFTR)
VKO123019836(CFTR)
PMUA114605368(CFTR)
PRAF128422198(CFTR)
ZVI118090952(CFTR)
HCG128326610(CFTR)
GJA107114507(CFTR)
STOW125434845(CFTR)
EMC129336023(CFTR)
XLA108712361(cftr.S) 373725(cftr.L)
XTR100486085(cftr)
NPR108802076(CFTR)
RTEM120931865(CFTR)
BBUF120981745(CFTR)
BGAR122927944(CFTR)
MUO115478815(CFTR)
GSH117367004(CFTR)
DRE559080(cftr)
SRX107708216 107738689
SANH107660943(cftr)
SGH107583749 107596947
CCAR109110485(cftr) 109110487
CAUA113070233 113118390
CGIB127934455 127977569
PTET122362859(cftr)
LROH127180588(cftr)
OMC131524216(cftr)
PPRM120487626(cftr)
RKG130088066(cftr)
MAMB125254502(cftr)
CIDE127500183
MASI127417053 127455661
TROS130562817(cftr)
TDW130435218(cftr)
MANU129418563(cftr)
IPU108264684
IFU128606734(cftr)
PHYP113534512(cftr)
SMEO124396118(cftr)
TFD113652314(cftr)
TVC132851213(cftr)
AMEX103032690(cftr)
CMAO118817736(cftr)
EEE113573602(cftr)
CHAR105889715(cftr)
TRU492310(cftr)
TFS130535948(cftr)
TNGGSTEN00029830G001
LCO104925520(cftr)
TBEN117479145(cftr)
CGOB115009192(cftr)
PGEO117447553(cftr)
GACU117541242(cftr)
ELY117262674(cftr)
EFO125886735(cftr)
PLEP121950083(cftr)
SLUC116036250(cftr)
ECRA117949343(cftr)
ESP116693709(cftr)
PFLV114559516(cftr)
GAT120809893(cftr)
PPUG119196655(cftr)
AFB129108095(cftr)
CLUM117731947(cftr)
MSAM119893949(cftr)
SCHU122874653(cftr)
CUD121515150(cftr)
ALAT119024427(cftr)
MZE101473042(cftr)
ONL100710776(cftr)
OAU116317517(cftr)
OLA101161380(cftr)
OML112139207(cftr)
XMA102231389(cftr)
XCO114143601(cftr)
XHE116709457(cftr)
PRET103465638(cftr)
PFOR103149394(cftr)
PLAI106953494(cftr)
PMEI106931684(cftr)
GAF122836074(cftr)
PPRL129353378(cftr)
CVG107083207(cftr)
CTUL119780462(cftr)
GMU124858225(cftr)
NFU107388351(cftr)
KMR108239387(cftr)
ALIM106518846(cftr)
NWH119415888(cftr)
AOCE111570180(cftr)
MCEP125015320(cftR)
CSEM103379580(cftr)
POV109624104(cftr)
SSEN122776327(cftr)
HHIP117763002(cftr)
HSP118110114(cftr)
LCF108893910
SDU111220518(cftr)
SLAL111672163(cftr)
XGL120793126(cftr)
HCQ109530830(cftr)
SSCV125970848
SBIA133501681(cftr)
PEE133403280(cftr)
PTAO133478148(cftr)
BPEC110155111(cftr)
MALB109956423(cftr)
BSPL114857513(cftr)
SJO128358564(cftr)
SASA100136364 100136366
STRU115152318 115197540
OTW112219020 112252645
OMY110499460 110532369
OGO124003923 124033673(cftr)
ONE115109658 115124429
OKI109867022 109889621
OKE118396051 118400975
SALP111952234 111962718
SNH120053520 120066717
CCLU121531542 121561492
ELS105029518(cftr)
SFM108931616(cftr)
PKI111842430(cftr)
AANG118219051 118232125(cftr)
LOC102683805(cftr)
PSPA121318222 121319888
ARUT117415934 117419968
PSEX120534116(cftr)
LCM102346483(CFTR)
CMK103179362(cftr)
RTP109925496(cftr)
CPLA122559692(cftr)
HOC132824686(cftr)
LERI129706505(cftr)
PMRN116951316(CFTR)
LRJ133356566 133356597
ADR102678461
CSEC111864333
XEN124446350
 » 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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