Protein Details: Cystic fibrosis transmembrane conductance regulator

Protein ID

ICDB_Pro_0442

Protein Name

Cystic fibrosis transmembrane conductance regulator

Gene Name

CFTR; ABCC7; DFTR

Organism

Squalus acanthias (Spiny dogfish)

Length

1492 amino acids

AlphaFoldDB

AF-P26362-F1-model_v4.pdb

Function

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis. Mediates the transport of chloride ions across the cell membrane (By similarity). Channel activity is coupled to ATP hydrolysis. The ion channel is also permeable to HCO(3)(-); selectivity depends on the extracellular chloride concentration. Exerts its function also by modulating the activity of other ion channels and transporters. Contributes to the regulation of the pH and the ion content of the epithelial fluid layer (By similarity).

Sequence

MQRSPIEKANAFSKLFFRWPRPILKKGYRQKLELSDIYQIPSSDSADELSEMLEREWDRELATSKKNPKLVNALRRCFFWRFLFYGILLYFVEFTKAVQPLCLGRIIASYNAKNTYEREIAYYLALGLCLLFVVRTLFLHPAVFGLQHLGMQMRIALFSLIYKKILKMSSRVLDKIDTGQLVSLLSNNLNKFDEGVAVAHFVWIAPVQVVLLMGLIWNELTEFVFCGLGFLIMLALFQAWLGKKMMQYRDKRAGKINERLAITSEIIDNIQSVKVYCWEDAMEKIIDDIRQVELKLTRKVAYCRYFSSSAFFFSGFFVVFLSVVPYAFIHTIKLRRIFTTISYNIVLRMTVTRQFPSAIQTWYDSLGAIRKIQDFLHKDEHKTVEYNLTTKEVEMVNVTASWDEGIGELFEKVKQNDSERKMANGDDGLFFSNFSLHVTPVLKNISFKLEKGELLAIAGSTGSGKSSLLMMIMGELEPSDGKIKHSGRISYSPQVPWIMPGTIKDNIIFGLSYDEYRYTSVVNACQLEEDITVFPNKDKTVLGDGGITLSGGQRARISLARALYKDADLYLLDSPFSHLDVTTEKDIFESCLCKLMVNKTRILVTSKLEHLKKADKILLLHEGHCYFYGTFSELQGEKPDFSSQLLGSVHFDSFSAERRNSILTETFRRCSVSSGDGAGLGSYSETRKASFKQPPPEFNEKRKSSLIVNPITSNKKFSLVQTAMSYPQTNGMEDATSEPGERHFSLIPENELGEPTKPRSNIFKSELPFQAHRRQSVLALMTHSSTSPNKIHARRSAVRKMSMLSQTNFASSEIDIYSRRLSEDGSFEISEEINEEDLKECFADEEEIQNVTTTWSTYLRYVTTNRNLVFVLILCLVIFLAEVAASLAGLWIISGLAINTGSQTNDTSTDLSHLSVFSKFITNGSHYYIFYIYVGLADSFLALGVIRGLPLVHTLVTVSKDLHKQMLHSVLQGPMTAFNKMKAGRILNRFIKDTAIIDDMLPLTVFDFVQLILIVVGAICVVSVLQPYTLLAAIPVAVIFIMLRAYFLRTSQQLKQLESEARSPIFSHLITSLRGLWTVRAFGRQSYFETLFHKALNLHTANWFLYLSTLRWFQMRIDIVFVLFFIAVTFIAIATHDVGEGQVGIILTLAMNITSTLQWAVNSSIDVDGLMRSVSRVFKYIDIPPEGSETKNRHNANNPSDVLVIENKHLTKEWPSGGQMMVNNLTAKYTSDGRAVLQDLSFSVNAGQRVGLLGRTGAGKSTLLSALLRLLSTEGEIQIDGISWNSVSLQKWRKAFGVIPQKVFVFSGTFRKNLDPYEQWSDEEIWKVTEEVGLKSMIEQFPDKLNFVLVDGGYILSNGHKQLMCLARSILSKAKILLLDEPTAHLDPVTFQIIRKTLKHTFSNCTVILSEHRVEALLECQQFLVIEGCSVKQFDALQKLLTEASLFKQVFGHLDRAKLFTAHRRNSSKRKTRPKISALQEEAEEDLQETRL

PDB Structures

Ligand Binding

1. DICL_CP

2. DICL_Pep

Binding Site

BINDING 402; /ligand="ATP"; BINDING 435; /ligand="ATP"; BINDING 459..466; /ligand="ATP"; BINDING 494; /ligand="ATP"; BINDING 1229; /ligand="ATP"; BINDING 1254..1261; /ligand="ATP"

Disease

Location

Expressed in the rectal gland (at protein level)

DOI ID

10.1016/s0021-9258(18)54631-7