Q00553

PR
Gene name
CFTR (ABCC7)
Protein name
Cystic fibrosis transmembrane conductance regulator
Names
CFTR, ATP-binding cassette sub-family C member 7, Channel conductance-controlling ATPase, cAMP-dependent chloride channel
Species
Macaca mulatta (Rhesus macaque)
KEGG Pathway
mcc:574346
EC number
5.6.1.6: Enzymes altering polypeptide conformation or assembly
Protein Class

Descriptions

The autoinhibited protein was predicted that may have potential autoinhibitory elements via cis-regPred.

Autoinhibitory domains (AIDs)

617-868 (Predicted disordered autoinhibitory region) PR

Target domain

Relief mechanism

Assay

cis-regPred

Accessory elements

No accessory elements

References

Autoinhibited structure

Activated structure

1 structures for Q00553

Entry ID Method Resolution Chain Position Source
AF-Q00553-F1 Predicted AlphaFoldDB

No variants for Q00553

Variant ID(s) Position Change Description Diseaes Association Provenance
No variants for Q00553

No associated diseases with Q00553

9 regional properties for Q00553

Type Name Position InterPro Accession
domain ABC transporter-like, ATP-binding domain 423 - 646 IPR003439-1
domain ABC transporter-like, ATP-binding domain 1211 - 1444 IPR003439-2
domain AAA+ ATPase domain 450 - 639 IPR003593-1
domain AAA+ ATPase domain 1237 - 1419 IPR003593-2
domain ABC transporter type 1, transmembrane domain 82 - 350 IPR011527-1
domain ABC transporter type 1, transmembrane domain 860 - 1158 IPR011527-2
conserved_site ABC transporter-like, conserved site 548 - 562 IPR017871
domain CFTR regulator domain 639 - 849 IPR025837
domain Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette domain 1 389 - 670 IPR047082

Functions

Description
EC Number 5.6.1.6 Enzymes altering polypeptide conformation or assembly
Subcellular Localization
  • Apical cell membrane ; Multi-pass membrane protein
  • Early endosome membrane ; Multi-pass membrane protein
  • Cell membrane ; Multi-pass membrane protein
  • Recycling endosome membrane ; Multi-pass membrane protein
  • Endoplasmic reticulum membrane ; Multi-pass membrane protein
  • Nucleus
  • The channel is internalized from the cell surface into an endosomal recycling compartment, from where it is recycled to the cell membrane
  • In the oviduct and bronchus, detected on the apical side of epithelial cells, but not associated with cilia
  • In Sertoli cells, a processed product is detected in the nucleus
  • ER stress induces GORASP2-mediated unconventional (ER/Golgi-independent) trafficking of core-glycosylated CFTR to cell membrane
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

10 GO annotations of cellular component

Name Definition
apical plasma membrane The region of the plasma membrane located at the apical end of the cell.
chloride channel complex An ion channel complex through which chloride ions pass.
early endosome A membrane-bounded organelle that receives incoming material from primary endocytic vesicles that have been generated by clathrin-dependent and clathrin-independent endocytosis; vesicles fuse with the early endosome to deliver cargo for sorting into recycling or degradation pathways.
early endosome membrane The lipid bilayer surrounding an early endosome.
endoplasmic reticulum membrane The lipid bilayer surrounding the endoplasmic reticulum.
integral component of membrane The component of a membrane consisting of the gene products and protein complexes having at least some part of their peptide sequence embedded in the hydrophobic region of the membrane.
integral component of plasma membrane The component of the plasma membrane consisting of the gene products and protein complexes having at least some part of their peptide sequence embedded in the hydrophobic region of the membrane.
nucleus A membrane-bounded organelle of eukaryotic cells in which chromosomes are housed and replicated. In most cells, the nucleus contains all of the cell's chromosomes except the organellar chromosomes, and is the site of RNA synthesis and processing. In some species, or in specialized cell types, RNA metabolism or DNA replication may be absent.
plasma membrane The membrane surrounding a cell that separates the cell from its external environment. It consists of a phospholipid bilayer and associated proteins.
recycling endosome membrane The lipid bilayer surrounding a recycling endosome.

9 GO annotations of molecular function

Name Definition
ABC-type transporter activity Primary active transporter characterized by two nucleotide-binding domains and two transmembrane domains. Uses the energy generated from ATP hydrolysis to drive the transport of a substance across a membrane.
ATP binding Binding to ATP, adenosine 5'-triphosphate, a universally important coenzyme and enzyme regulator.
ATP hydrolysis activity Catalysis of the reaction: ATP + H2O = ADP + H+ phosphate. ATP hydrolysis is used in some reactions as an energy source, for example to catalyze a reaction or drive transport against a concentration gradient.
bicarbonate transmembrane transporter activity Enables the transfer of bicarbonate from one side of a membrane to the other. Bicarbonate is the hydrogencarbonate ion, HCO3-.
chloride channel activity Enables the facilitated diffusion of a chloride (by an energy-independent process) involving passage through a transmembrane aqueous pore or channel without evidence for a carrier-mediated mechanism.
chloride channel inhibitor activity Binds to and stops, prevents, or reduces the activity of a chloride channel.
chloride transmembrane transporter activity Enables the transfer of chloride ions from one side of a membrane to the other.
intracellularly ATP-gated chloride channel activity Enables passage of a chloride ion through a transmembrane channel that opens when ATP is bound by the channel complex or one of its constituent parts on the intracellular side of the plasma membrane.
isomerase activity Catalysis of the geometric or structural changes within one molecule. Isomerase is the systematic name for any enzyme of EC class 5.

10 GO annotations of biological process

Name Definition
bicarbonate transport The directed movement of bicarbonate into, out of or within a cell, or between cells, by means of some agent such as a transporter or pore.
cellular response to cAMP Any process that results in a change in state or activity of a cell (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a cAMP (cyclic AMP, adenosine 3',5'-cyclophosphate) stimulus.
cellular response to forskolin Any process that results in a change in state or activity of a cell (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a forskolin stimulus.
chloride transmembrane transport The process in which chloride is transported across a membrane.
intracellular pH elevation Any process that increases the internal pH of a cell, measured by the concentration of the hydrogen ion.
membrane hyperpolarization The process in which membrane potential increases with respect to its steady-state potential, usually from negative potential to a more negative potential. For example, during the repolarization phase of an action potential the membrane potential often becomes more negative or hyperpolarized before returning to the steady-state resting potential.
multicellular organismal water homeostasis Any process involved in the maintenance of an internal steady state of water within a tissue, organ, or a multicellular organism.
response to endoplasmic reticulum stress Any process that results in a change in state or activity of a cell (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a stress acting at the endoplasmic reticulum. ER stress usually results from the accumulation of unfolded or misfolded proteins in the ER lumen.
sperm capacitation A process required for sperm to reach fertilization competence. Sperm undergo an incompletely understood series of morphological and molecular maturational processes, termed capacitation, involving, among other processes, protein tyrosine phosphorylation and increased intracellular calcium.
transepithelial water transport The directed movement of water (H2O) from one side of an epithelium to the other.

26 homologous proteins in AiPD

UniProt AC Gene Name Protein Name Species Evidence Code
Q8HXQ5 ABCC1 Multidrug resistance-associated protein 1 Bos taurus (Bovine) PR
Q2QLE5 CFTR Cystic fibrosis transmembrane conductance regulator Pan troglodytes (Chimpanzee) PR
Q5U820 CFTR Cystic fibrosis transmembrane conductance regulator Canis lupus familiaris (Dog) (Canis familiaris) PR
P91660 Rh5 Probable multidrug resistance-associated protein lethal(2)03659 Drosophila melanogaster (Fruit fly) PR
Q2QLA3 CFTR Cystic fibrosis transmembrane conductance regulator Equus caballus (Horse) PR
O95255 ABCC6 ATP-binding cassette sub-family C member 6 Homo sapiens (Human) PR
O15438 ABCC3 ATP-binding cassette sub-family C member 3 Homo sapiens (Human) PR
Q92887 ABCC2 ATP-binding cassette sub-family C member 2 Homo sapiens (Human) SS
O15439 ABCC4 ATP-binding cassette sub-family C member 4 Homo sapiens (Human) PR
Q96J66 ABCC11 ATP-binding cassette sub-family C member 11 Homo sapiens (Human) PR
P33527 ABCC1 Multidrug resistance-associated protein 1 Homo sapiens (Human) PR
P13569 CFTR Cystic fibrosis transmembrane conductance regulator Homo sapiens (Human) PR
B2RX12 Abcc3 ATP-binding cassette sub-family C member 3 Mus musculus (Mouse) PR
Q8VI47 Abcc2 ATP-binding cassette sub-family C member 2 Mus musculus (Mouse) SS
O35379 Abcc1 Multidrug resistance-associated protein 1 Mus musculus (Mouse) PR
Q80WJ6 Abcc12 ATP-binding cassette sub-family C member 12 Mus musculus (Mouse) PR
P26361 Cftr Cystic fibrosis transmembrane conductance regulator Mus musculus (Mouse) PR
Q6PQZ2 CFTR Cystic fibrosis transmembrane conductance regulator Sus scrofa (Pig) PR
Q6Y306 Abcc12 ATP-binding cassette sub-family C member 12 Rattus norvegicus (Rat) PR
Q8CG09 Abcc1 Multidrug resistance-associated protein 1 Rattus norvegicus (Rat) PR
Q63120 Abcc2 ATP-binding cassette sub-family C member 2 Rattus norvegicus (Rat) EV
Q9C8G9 ABCC1 ABC transporter C family member 1 Arabidopsis thaliana (Mouse-ear cress) PR
Q9C8H0 ABCC12 ABC transporter C family member 12 Arabidopsis thaliana (Mouse-ear cress) PR
Q9SKX0 ABCC13 ABC transporter C family member 13 Arabidopsis thaliana (Mouse-ear cress) PR
Q8VZZ4 ABCC6 ABC transporter C family member 6 Arabidopsis thaliana (Mouse-ear cress) PR
Q9M1C7 ABCC9 ABC transporter C family member 9 Arabidopsis thaliana (Mouse-ear cress) PR
10 20 30 40 50 60
MQRSPLEKAS VVSKLFFSWT RPILRKGYRQ RLELSDIYQI PSADSADNLS EKLEREWDRE
70 80 90 100 110 120
LASKKNPKLI NALRRCFFWR FMFYGILLYL GEVTKAVQPL LLGRIIASYD PDNKEERSIA
130 140 150 160 170 180
IYLGIGLCLL FIVRTLLLHP AIFGLHHIGM QMRIAMFSLI YKKTLKLSSR VLDKISIGQL
190 200 210 220 230 240
VSLLSNNLNK FDEGLALAHF VWIVPLQVAL LMGLIWELLQ ASAFCGLGFL IVLALFQAGL
250 260 270 280 290 300
GRMMMKYRDQ RAGKINERLV ITSEMIENIQ SVKAYCWEEA MEKMIENLRQ TELKLTRKAA
310 320 330 340 350 360
YVRYFNSSAF FFSGFFVVFL SVLPYALIKG IVLRKIFTTI SFCIVLRMAV TRQFPWAVQT
370 380 390 400 410 420
WYDSLGAINK IQDFLQKQEY KTLEYNLTTT EVVMENVTAF WEEGFGELFE KAKQNNSNRK
430 440 450 460 470 480
TSNDDDSLFF SNFSLLGTPV LKDINFKIER GQLLAVAGST GAGKTSLLMM IMGELEPSEG
490 500 510 520 530 540
KIKHSGRISF CSQFSWIMPG TIKENIIFGV SYDEYRYRSV INACQLEEDI SKFAEKDNIV
550 560 570 580 590 600
LGEGGITLSG GQRARISLAR AVYKDADLYL LDSPFGYLDV LTEKEIFESC VCKLMANKTR
610 620 630 640 650 660
ILVTSKMEHL KKADKILILH EGSSYFYGTF SELQNLRPDF SSKLMGYDSF DQFSAERRNS
670 680 690 700 710 720
ILTETLRRFS LEGDAPVSWT ETKKQSFKQT GEFGEKRKNS ILNPINSIRK FSIVQKTPLQ
730 740 750 760 770 780
MNGIEEDSDE PLERRLSLVP DSEQGEVILP RISVISTGPT LQARRRQSVL NLMTHSVNQG
790 800 810 820 830 840
QSIHRKTAAS TRKVSLAPQA NLTELDIYSR RLSQETGLEI SEEINEEDLK ECFFDDMESI
850 860 870 880 890 900
PAVTTWNTYL RYITVHKSLI FVLIWCLVIF LAEVAASLVV LWFLGNTPPQ DKGNSTYSRN
910 920 930 940 950 960
NSYAVIITRT SSYYVFYIYV GVADTLLAMG FFRGLPLVHT LITVSKILHH KMLHSVLQAP
970 980 990 1000 1010 1020
MSTLNTLKAG GILNRFSKDI AILDDLLPLT IFDFIQLLLI VIGAIAVVAV LQPYIFVATV
1030 1040 1050 1060 1070 1080
PVIVAFIMLR AYFLQTSQQL KQLESEGRSP IFTHLVTSLK GLWTLRAFGR QPYFETLFHK
1090 1100 1110 1120 1130 1140
ALNLHTANWF LYLSTLRWFQ MRIEMIFVIF FIAVTFISIL TTGEGEGTVG IILTLAMNIM
1150 1160 1170 1180 1190 1200
STLQWAVNSS IDVDSLMRSV SRVFKFIDMP TEEGKPTRST KPYKNGQLSK VMVIENSHVK
1210 1220 1230 1240 1250 1260
KDDIWPSGGQ MTVKDLTAKY TEGGNPILEN ISFSISPGQR VGLLGRTGSG KSTLLSAFLR
1270 1280 1290 1300 1310 1320
LLNTEGEIQI DGVSWDSITL QQWRKAFGVI PQKVFIFSGT FRKNLDPYEQ WSDQEIWKVA
1330 1340 1350 1360 1370 1380
DEVGLRSVIE QFPGKLDFVL VDGGCVLSHG HKQLMCLARS VLSKAKILLL DEPSAHLDPV
1390 1400 1410 1420 1430 1440
TYQIIRRTLK QAFADCTVIL CEHRIEAMLE CQQFLVIEEN KVRQYDSIQK LLNERSLFRQ
1450 1460 1470 1480
AISPSDRVKL FPHRNSSKCK TQPQIAALKE ETEEEVQDTR L