CLCN3

2Cl^-/H⁺ exchanger 3 is a protein that in humans is encoded by the CLCN3 gene.^[5]

CLCN3
Identifiers
Aliases	CLCN3, CLC3, ClC-3, chloride voltage-gated channel 3, NEDSBA, NEDHYBA
External IDs	OMIM: 600580; MGI: 103555; HomoloGene: 20435; GeneCards: CLCN3; OMA:CLCN3 - orthologs
Gene location (Human) Chr. Chromosome 4 (human)[1] Band 4q33 Start 169,612,633 bp[1] End 169,723,673 bp[1]
Gene location (Mouse) Chr. Chromosome 8 (mouse)[2] Band 8 B3.1|8 30.9 cM Start 61,363,423 bp[2] End 61,436,334 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in mucosa of sigmoid colon endothelial cell Brodmann area 46 Epithelium of choroid plexus retinal pigment epithelium postcentral gyrus islet of Langerhans parotid gland right ventricle superior vestibular nucleus Top expressed in Rostral migratory stream mammillary body lateral septal nucleus ventromedial nucleus lateral geniculate nucleus lateral hypothalamus cingulate gyrus anterior amygdaloid area paraventricular nucleus of hypothalamus subiculum More reference expression data BioGPS More reference expression data
Gene ontology Molecular function nucleotide binding volume-sensitive chloride channel activity PDZ domain binding protein homodimerization activity ion channel activity protein binding protein heterodimerization activity antiporter activity ATP binding chloride channel activity voltage-gated chloride channel activity solute:proton antiporter activity chloride ion binding Cellular component integral component of membrane endosome late endosome Golgi apparatus early endosome membrane membrane late endosome membrane synaptic vesicle vesicle membrane Golgi membrane plasma membrane transport vesicle membrane secretory granule cell surface early endosome specific granule phagocytic vesicle external side of plasma membrane cytoplasmic vesicle integral component of plasma membrane Biological process negative regulation of cell volume endosomal lumen acidification ion transport regulation of anion transmembrane transport chloride transport regulation of pH chloride transmembrane transport regulation of anion transport transmembrane transport transport synaptic vesicle lumen acidification Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 1182 12725 Ensembl ENSG00000109572 ENSMUSG00000004319 UniProt P51790 P51791 RefSeq (mRNA) NM_001243372 NM_001243374 NM_001829 NM_173872 NM_007711 NM_173873 NM_173874 NM_173876 RefSeq (protein) NP_001230301 NP_001230303 NP_001820 NP_776297 NP_031737 NP_776298 NP_776299 NP_776301 Location (UCSC) Chr 4: 169.61 – 169.72 Mb Chr 8: 61.36 – 61.44 Mb PubMed search [3] [4]
Wikidata
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Interactions

CLCN3 has been shown to interact with PDZK1 and TMEM9.<ref name=pmid12471024>Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR (Feb 2003). "The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteins". J. Biol. Chem. 278 (8): 6440–9. doi:10.1074/jbc.M211050200. PMID 12471024.<pmid40169677>Planells-Cases R, Vorobeva V, Kar S, Schmitt F, Schulte U, Schrecker M, Hite R, Fakler B, Jentsch T (2025). "Endosomal chloride/proton exchangers need inhibitory TMEM9 β-subunits for regulation and prevention of disease-causing overactivity". Nature Communications. 16: 1–17. doi:10.1038/s41467-025-58546-3. PMID 40169677.

See also

• Chloride channel