KEGG ENZYME: 5.6.1.6

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)

PPYG:

129040730(CFTR)

NLE:	100601480(CFTR)

HMH:	116810950(CFTR)

SSYN:

129484923(CFTR)

MCC:	574346(CFTR)

MCF:	102130331(CFTR)

MTHB:

126951193

MNI:	105475504(CFTR)

CSAB:

103226777(CFTR)

CATY:

105598482(CFTR)

PANU:

100126675(CFTR)

TGE:	112620581(CFTR)

MLEU:

105533272(CFTR)

RRO:	104682625(CFTR)

RBB:	108526537(CFTR)

TFN:	117072767(CFTR)

PTEH:

111553406(CFTR)

CANG:

105508520(CFTR)

CJC:	100415251(CFTR)

SBQ:	101036459(CFTR)

CIMI:

108308994(CFTR)

ANAN:

105733776(CFTR)

CSYR:

103264109(CFTR)

MMUR:

105869370(CFTR)

LCAT:

123646968(CFTR)

PCOQ:

105817115(CFTR)

OGA:	100961312(CFTR)

MMU:	12638(Cftr)

MCAL:

110296314(Cftr)

MPAH:

110316437(Cftr)

RNO:	24255(Cftr)

MCOC:

116098609(Cftr)

ANU:	117720697(Cftr)

MUN:	110564980(Cftr)

CGE:	100754506(Cftr)

MAUA:

101831516(Cftr)

PROB:

127215341(Cftr)

PLEU:

114694716(Cftr)

MORG:

121463441(Cftr)

MFOT:

126503972

AAMP:

119807517(Cftr)

NGI:	103737361(Cftr)

HGL:	101709472(Cftr)

CPOC:

100719898(Cftr)

CCAN:

109679300(Cftr)

DORD:

105988326(Cftr)

DSP:	122121761(Cftr)

PLOP:

125345484(Cftr)

NCAR:

124991796

MMMA:

107144405(Cftr)

OCU:	100009471(CFTR)

OPI:	101535310(CFTR)

TUP:	102490714(CFTR)

GVR:	103597253(CFTR)

CFA:	492302(CFTR)

CLUD:

112670805(CFTR)

VVP:	112929896(CFTR)

VLG:	121475063(CFTR)

NPO:	129502846(CFTR)

AML:	100478723(CFTR)

UMR:	103676590(CFTR)

UAH:	113263308(CFTR)

UAR:	123789030(CFTR)

ELK:

111152043

LLV:

125081083

MPUF:

101672484(CFTR)

MNP:	132015768(CFTR)

MLK:	131829662(CFTR)

NVS:	122905012(CFTR)

ORO:	101363648(CFTR)

EJU:	114210604(CFTR)

ZCA:	113911768(CFTR)

MLX:	118020550(CFTR)

NSU:	110588952(CFTR)

LWW:	102736525(CFTR)

FCA:	492308(CFTR)

PYU:	121028439(CFTR)

PCOO:

112866723(CFTR)

PBG:	122488303(CFTR)

PVIV:

125160811(CFTR)

LRUF:

124524870

PTG:	102956215(CFTR)

PPAD:

109248378(CFTR)

PUC:

125930080

AJU:

106970664

HHV:

120224616

BTA:	281067(CFTR)

BOM:	102264773(CFTR)

BIU:	109557891(CFTR)

BBUB:

102397474(CFTR)

BBIS:

104981947(CFTR)

CHX:	102179446(CFTR)

OAS:	443347(CFTR)

BTAX:

128046774(CFTR)

ODA:	120877516(CFTR)

CCAD:

122438337(CFTR)

MBEZ:

129552417(CFTR)

SSC:	403154(CFTR)

CFR:	102505598(CFTR)

CBAI:

105063144(CFTR)

CDK:	105085325(CFTR)

VPC:	102527896(CFTR)

BACU:

103004581(CFTR)

BMUS:

118901296(CFTR)

LVE:	103088529(CFTR)

OOR:	101278319(CFTR)

DLE:	111179250(CFTR)

PCAD:

102973776(CFTR)

PSIU:

116759844(CFTR)

NASI:

112408186(CFTR)

ECB:	100071259(CFTR)

EPZ:	103550601(CFTR)

EAI:	106829379(CFTR)

MYB:	102257625(CFTR)

MYD:	102773631(CFTR)

MMYO:

118666477(CFTR)

MLF:	102419125(CFTR)

MDT:	132242677(CFTR)

PKL:	118711824(CFTR)

EFUS:

103295955(CFTR)

MNA:	107524880(CFTR)

DRO:	112300407(CFTR)

SHON:

118999581(CFTR)

AJM:	119035603(CFTR)

PDIC:

114507755(CFTR)

PHAS:

123824765(CFTR)

MMF:	118619948(CFTR)

PPAM:

129063035(CFTR)

HAI:

109376431

RFQ:	117018340(CFTR)

PALE:

102884577(CFTR)

PGIG:

120601125(CFTR)

PVP:	105308468(CFTR)

RAY:	107519971(CFTR)

MJV:	108387496(CFTR)

TOD:	119250439(CFTR)

SARA:

101556770(CFTR)

SETR:

126016360(CFTR)

LAV:	100662653(CFTR)

TMU:

101346801

ETF:	101657112(CFTR)

DNM:	101433838(CFTR)

MDO:	100014119(CFTR)

GAS:	123249455(CFTR)

SHR:	100919086(CFTR)

AFZ:

127538624

PCW:	110200665(CFTR)

TVP:	118851838(CFTR)

OAA:	100078764(CFTR)

GGA:	100049619(CFTR)

PCOC:

116243921(CFTR)

MGP:	100545354(CFTR)

CJO:	107309076(CFTR)

TPAI:

128073470(CFTR)

LMUT:

125694041(CFTR)

NMEL:

110392590(CFTR)

APLA:

101803386(CFTR)

ACYG:

106042053(CFTR)

CATA:

118246773(CFTR)

AFUL:

116501959(CFTR)

TGU:	100222243(CFTR)

LSR:	110483509(CFTR)

SCAN:

103813684(CFTR)

PMOA:

120510781(CFTR)

OTC:	121344821(CFTR)

PRUF:

121355836(CFTR)

GFR:	102041165(CFTR)

FAB:	101809710(CFTR)

OMA:	130248254(CFTR)

PHI:	102114534(CFTR)

PMAJ:

107205064(CFTR)

CCAE:

111937028(CFTR)

CCW:	104698445(CFTR)

CBRC:

103614852(CFTR)

ETL:	114062358(CFTR)

ZAB:	102064789(CFTR)

ZLE:	135457804(CFTR)

ACHL:

103799685(CFTR)

SVG:	106862907(CFTR)

MMEA:

130589221(CFTR)

HRT:	120751779(CFTR)

SATI:

136361223(CFTR)

FPG:	101921929(CFTR)

FCH:	102050923(CFTR)

CCRI:

104161185(CFTR)

NNT:	104409589(CFTR)

SHAB:

115604828(CFTR)

ACUN:

113491166(CFTR)

TALA:

104355842(CFTR)

ACHC:

115342266(CFTR)

HALD:

104317503

HLE:	104835814(CFTR)

AGEN:

126044249

GCL:

127022852

CSTI:

104559106(CFTR)

LDI:	104351295(CFTR)

MNB:	103778346(CFTR)

DPUB:

104309179(CFTR)

AVIT:

104272747

BRHI:

104494230(CFTR)

EGZ:	104133415(CFTR)

NNI:	104013586(CFTR)

PCRI:

104029724(CFTR)

PCAO:

104049962(CFTR)

PADL:

103917004(CFTR)

AFOR:

103905223(CFTR)

FGA:

104081922

GSTE:

104256497(CFTR)

CLV:	102089481(CFTR)

MUI:	104537232(CFTR)

PGUU:

104472123(CFTR)

PLET:

104627445(CFTR)

EHS:	104506564(CFTR)

CMAC:

104473521(CFTR)

CUCA:

104064321(CFTR)

TEO:	104377114(CFTR)

BREG:

104636612(CFTR)

OHA:	104337503(CFTR)

ACAR:

104522661(CFTR)

CPEA:

104394692(CFTR)

CVF:	104285252(CFTR)

RTD:	128904165(CFTR)

AAM:	106488125(CFTR)

AROW:

112965500(CFTR)

NPD:	112942325(CFTR)

TGT:	104580765(CFTR)

DNE:	112981586(CFTR)

SCAM:

104153576(CFTR)

ASN:	102369471(CFTR)

AMJ:	102563163(CFTR)

CPOO:

109315988(CFTR)

GGN:	109296354(CFTR)

PSS:	102447829(CFTR)

CMY:	102936252(CFTR)

CCAY:

125636148(CFTR)

DCC:	119850071(CFTR)

CPIC:

101933840(CFTR)

TST:	117870523(CFTR)

CABI:

116838613(CFTR)

MRV:	120388529(CFTR)

ACS:	100559531(cftr)

ASAO:

132776261(CFTR)

PVT:	110077041(CFTR)

SUND:

121932080(CFTR)

PBI:	103057487(CFTR)

PMUR:

107285369(CFTR)

CTIG:

120308230(CFTR)

TSR:	106538296(CFTR)

PGUT:

117673597(CFTR)

APRI:

131202459(CFTR)

PTEX:

113444417(CFTR)

NSS:	113413065(CFTR)

VKO:	123019836(CFTR)

PMUA:

114605368(CFTR)

PRAF:

128422198(CFTR)

ZVI:	118090952(CFTR)

HCG:	128326610(CFTR)

GJA:	107114507(CFTR)

STOW:

125434845(CFTR)

EMC:	129336023(CFTR)

XLA:	108712361(cftr.S) 373725(cftr.L)

XTR:	100486085(cftr)

NPR:	108802076(CFTR)

RTEM:

120931865(CFTR)

BBUF:

120981745(CFTR)

BGAR:

122927944(CFTR)

MUO:	115478815(CFTR)

GSH:	117367004(CFTR)

DRE:	559080(cftr)

SRX:	107708216 107738689

SANH:

107660943(cftr)

SGH:	107583749 107596947

CCAR:

109110485(cftr) 109110487

CAUA:

113070233 113118390

CGIB:

127934455 127977569

PTET:

122362859(cftr)

LROH:

127180588(cftr)

OMC:	131524216(cftr)

PPRM:

120487626(cftr)

RKG:	130088066(cftr)

MAMB:

125254502(cftr)

CIDE:

127500183

CERY:

137023368(cftr)

MASI:

127417053 127455661

TROS:

130562817(cftr)

TDW:	130435218(cftr)

MANU:

129418563(cftr)

IPU:

108264684

IFU:	128606734(cftr)

PHYP:

113534512(cftr)

SMEO:

124396118(cftr)

TFD:	113652314(cftr)

TVC:	132851213(cftr)

TRN:	134323177(cftr)

AMEX:

103032690(cftr)

CMAO:

118817736(cftr)

EEE:	113573602(cftr)

CHAR:

105889715(cftr)

TRU:	492310(cftr)

TFS:	130535948(cftr)

TNG:	GSTEN00029830G001

LCO:	104925520(cftr)

TBEN:

117479145(cftr)

CGOB:

115009192(cftr)

PGEO:

117447553(cftr)

GACU:

117541242(cftr)

EMAC:

134881129(cftr)

ELY:	117262674(cftr)

EFO:	125886735(cftr)

PLEP:

121950083(cftr)

SLUC:

116036250(cftr)

ECRA:

117949343(cftr)

ESP:	116693709(cftr)

PFLV:

114559516(cftr)

GAT:	120809893(cftr)

PPUG:

119196655(cftr)

AFB:	129108095(cftr)

CLUM:

117731947(cftr)

PSWI:

130194626(cftr)

MSAM:

119893949(cftr)

SCHU:

122874653(cftr)

CUD:	121515150(cftr)

ALAT:

119024427(cftr)

MZE:	101473042(cftr)

ONL:	100710776(cftr)

OAU:	116317517(cftr)

OLA:	101161380(cftr)

OML:	112139207(cftr)

CSAI:

133443740(cftr)

XMA:	102231389(cftr)

XCO:	114143601(cftr)

XHE:	116709457(cftr)

PRET:

103465638(cftr)

PFOR:

103149394(cftr)

PLAI:

106953494(cftr)

PMEI:

106931684(cftr)

GAF:	122836074(cftr)

PPRL:

129353378(cftr)

CVG:	107083207(cftr)

CTUL:

119780462(cftr)

GMU:	124858225(cftr)

NFU:	107388351(cftr)

KMR:	108239387(cftr)

ALIM:

106518846(cftr)

NWH:	119415888(cftr)

AOCE:

111570180(cftr)

MCEP:

125015320(cftR)

CSEM:

103379580(cftr)

POV:	109624104(cftr)

SSEN:

122776327(cftr)

HHIP:

117763002(cftr)

HSP:	118110114(cftr)

PPLT:

128444938(cftr)

SMAU:

118314811(cftr)

LCF:

108893910

SDU:	111220518(cftr)

SLAL:

111672163(cftr)

XGL:	120793126(cftr)

HCQ:	109530830(cftr)

SSCV:

125970848

SBIA:

133501681(cftr)

PEE:	133403280(cftr)

PTAO:

133478148(cftr)

BPEC:

110155111(cftr)

MALB:

109956423(cftr)

BSPL:

114857513(cftr)

SJO:	128358564(cftr)

SASA:

100136364 100136366

STRU:

115152318 115197540

OTW:	112219020 112252645

OMY:	110499460 110532369

OGO:	124003923 124033673(cftr)

ONE:	115109658 115124429

OKI:	109867022 109889621

OKE:	118396051 118400975

SALP:

111952234 111962718

SNH:	120053520 120066717

CCLU:

121531542 121561492

ELS:	105029518(cftr)

SFM:	108931616(cftr)

PKI:	111842430(cftr)

AANG:

118219051 118232125(cftr)

LOC:	102683805(cftr)

PSPA:

121318222 121319888

ARUT:

117415934 117419968

PSEX:

120534116(cftr)

LCM:	102346483(CFTR)

CMK:	103179362(cftr)

RTP:	109925496(cftr)

CPLA:

122559692(cftr)

HOC:	132824686(cftr)

LERI:

129706505(cftr)

PMRN:

116951316(CFTR)

LRJ:	133356566 133356597

ADR:

102678461

CSEC:

111864333

XEN:

124446350

» 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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