PF14396

Cystic fibrosis TM conductance regulator (CFTR), regulator domain

Pfam entry
Member databasePfam
Pfam typedomain
Short nameCFTR_R
ClanP-loop_NTPase
Author Finn RD;0000-0001-8626-2148
Sequence Ontology0000417

Description
Imported from IPR025837

Cystic fibrosis transmembrane conductance regulator (CFTR) that belongs to the ATP-binding cassette (ABC) transporter superfamily. It is a member of the ABC-C subfamily, which also contains the SUR receptors and the multidrug- resistance associated proteins (MRP)
[4]
. The CFTR protein encodes a chloride ion channel, which is controlled by phosphorylation. It has a major role in electrolyte and fluid secretion. CFTR is important in the determination of fluid flow, ion concentration and transepithelial salt transport. Dysfunction of the CFTR channel causes the life-threatening disease, cystic fibrosis, in which trans-epithelial ion transport is disrupted
[5]
.

This entry represents the CFTR regulator domain
[1, 3, 2]
.

References
Imported from IPR025837

1.Phosphorylation of CFTR by PKA promotes binding of the regulatory domain. Chappe V, Irvine T, Liao J, Evagelidis A, Hanrahan JW. EMBO J. 24, 2730-40, (2005). View articlePMID: 16001079

2.The inhibition mechanism of non-phosphorylated Ser768 in the regulatory domain of cystic fibrosis transmembrane conductance regulator. Wang G. J. Biol. Chem. 286, 2171-82, (2011). View articlePMID: 21059651

3.CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices. Baker JM, Hudson RP, Kanelis V, Choy WY, Thibodeau PH, Thomas PJ, Forman-Kay JD. Nat. Struct. Mol. Biol. 14, 738-45, (2007). View articlePMID: 17660831

4.The human ATP-binding cassette (ABC) transporter superfamily. Dean M, Rzhetsky A, Allikmets R. Genome Res. 11, 1156-66, (2001). View articlePMID: 11435397

5.Structure and function of the CFTR chloride channel. Sheppard DN, Welsh MJ. Physiol. Rev. 79, S23-45, (1999). View articlePMID: 9922375

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