chloride channel protein ClC-Kb; chloride channel Kb; ClC-K2 (CLCNKB)

Function: - voltage-gated chloride channel - chloride channels have several functions including a) regulation of cell volume b) membrane potential stabilization c) signal transduction d) transepithelial transport - may be important in urinary concentrating mechanisms - interacts with barttin (BSND) - forms heteromers with BSND in the thick ascending limb of Henle & more distal segments Structure: - belongs to the chloride channel (TC 2.A.49) family - contains 2 CBS domains Compartment: membrane Expression: expressed predominantly in the kidney Pathology: - defects in CLCNKB are the cause of Bartter syndrome type 3 Notes: - compared with CLCNKA/BSND, CLCNKB/BSND is more sensitive to pH & less responsive to Ca⁺²

General

Cl- channel glycoprotein transmembrane 12 protein

Properties

SIZE: entity length = 687 aa MW = 75 kD COMPARTMENT: plasma membrane MOTIF: cytoplasmic domain {1-49} transmembrane domain {50-69} exoplasmic loop {70-90} transmembrane domain {91-114} cytoplasmic loop {115-137} transmembrane domain {138-159} exoplasmic loop {160-168} transmembrane domain {169-188} cytoplasmic loop {189-199} transmembrane domain {200-224} exoplasmic loop {225-238} transmembrane domain {239-257} cytoplasmic loop {258-281} transmembrane domain {282-302} exoplasmic loop {303-324} transmembrane domain {325-348} cytoplasmic loop {349-397} transmembrane domain {398-417} exoplasmic loop {418-419} transmembrane domain {420-438} cytoplasmic loop {439-465} transmembrane domain {466-487} exoplasmic loop {488-494} transmembrane domain {495-514} cytoplasmic loop {515-645} MOTIF: CBS 1 {551-609} CBS 2 {626-684} MOTIF: transmembrane domain {646-664} exoplasmic domain {665-687} MOTIF: N-glycosylation site {N679} ION-PERMEABILITY: Cl-

Database Correlations

OMIM correlations MORBIDMAP 602023 UniProt P51801 PFAM correlations Entrez Gene 1188 Kegg hsa:1188

References

UniProt :accession P51801