CLCNKA

Chr 1p36.13

chloride voltage-gated channel Ka

Aliases:: hClC-Ka, ClC-K1, CLCK1
MANE:: ENST00000331433.5

OMIM: 602024 ↗NCBI: 1187 ↗Ensembl ↗

Annotations refreshed 10 hours ago.

Predicted protein structure

AlphaFold predicted structure

CLCNKA · P51800

v62025-08-01

Predicted Aligned Error matrix for CLCNKA — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

87.1/ 100

Confident

687 residues

Confidence breakdown

Very high(≥ 90)59%
Confident(70–90)33%
Low(50–70)5%
Very low(< 50)4%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Fetal anomalies
BIALLELIC, autosomal or pseudoautosomal
DDG2P
BIALLELIC, autosomal or pseudoautosomal
Intellectual disability
Other
Nephrocalcinosis or nephrolithiasis
Other
Renal tubulopathies
Other

Disease associations (Open Targets)

Bartter syndrome
0.59
Bartter syndrome type 4
0.41
neurodegenerative disease
0.41
heart failure
0.41
cardiomyopathy
0.41
systolic heart failure
0.40
deafness
0.40
cardioverter defibrillator
0.37
congestive heart failure
0.36
hypertrophic cardiomyopathy
0.35

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Chloride channel protein ClC-Ka

Anion-selective channel permeable to small monovalent anions with ion selectivity for chloride > bromide > nitrate > iodide (PubMed:11734858, PubMed:12111250). Forms a homodimeric channel where each subunit has its own ion conduction pathway. May conduct double-barreled currents controlled by two types of gates, two fast gates that control each subunit independently and a slow common gate that opens and shuts off both subunits simultaneously (PubMed:11734858, PubMed:12111250, PubMed:18310267, PubMed:18776122, PubMed:19646679, PubMed:20538786). Assembles with the regulatory subunit BSND/Barttin for sorting at the basolateral plasma membrane domain and functional switch to the ion conducting state. CLCNKA:BSND channels display mostly a linear current-voltage relationship with fast gating at negative potentials (PubMed:11734858, PubMed:12111250, PubMed:18310267, PubMed:18776122, PubMed:20538786). Mediates transepithelial chloride transport from the lumen to interstitial compartment along the thin ascending limb of Henle's loop, contributing to generation of hypertonic medullary interstitium as a countercurrent system to achieve urine concentration (By similarity) (PubMed:15044642). Conducts chloride currents in the stria vascularis of the inner ear to establish the endocochlear potential necessary for normal hearing (PubMed:15044642, PubMed:18310267, PubMed:19646679)