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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KCNJ12
potassium inwardly rectifying channel subfamily J member 12
Chromosome 17 · 17p11.2
NCBI Gene: 3768Ensembl: ENSG00000184185.11HGNC: HGNC:6258UniProt: Q14500
49PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Ion ChannelTransporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
inward rectifier potassium channel activityprotein bindingpotassium ion transportprotein homotetramerizationHypercholesterolemiatype 2 diabetes mellitusneurodegenerative diseasehealth study participation
✦AI Summary

KCNJ12 encodes Kir2.2, an inward rectifying potassium channel that controls resting membrane potential in electrically excitable tissues 1. The channel allows preferential potassium influx, with inward rectification primarily mediated by internal magnesium blockade of outward current 2. KCNJ12 is located on chromosome 17.1 as a single-exon gene in humans 1, though canine ortholog analysis revealed conserved three-exon structure across vertebrates 3. The arginine residue at position 285 in the C-terminal domain is critical for functional expression in mammalian cells 2. Pathologically, KCNJ12 mutations cause dilated cardiomyopathy (DCM), with a KCNJ12 p.Glu334del mutation identified in a family presenting with heart failure, arrhythmia, and sudden death 4. Additionally, rare germline KCNJ12/KCNJ18 mutations associate with familial esophageal squamous cell carcinoma risk 5. Beyond Mendelian disorders, KCNJ12 expression is regulated in cancer: circRNA hsa_circ_0014130 acts as a miR-132-3p sponge to upregulate KCNJ12 in bladder cancer, promoting epithelial-mesenchymal transition and GSK3β/AKT signaling 6. KCNJ12 DNA methylation status serves as a novel biomarker for colorectal cancer detection in blood with high diagnostic sensitivity 7.

Sources cited
1
KCNJ12 gene structure, chromosomal location at 17p11.1, and characterization as single-exon gene
PMID: 9027495
2
Arginine 285 residue importance for KCNJ12 functional expression in mammalian cells
PMID: 12417321
3
Comparative KCNJ12 gene structure conservation across vertebrates and functional properties of canine Kir2.2
PMID: 22363290
4
KCNJ12 p.Glu334del mutation causes familial dilated cardiomyopathy with arrhythmia and sudden death
PMID: 28816949
5
Rare germline KCNJ12/KCNJ18 mutations associated with familial esophageal squamous cell carcinoma
PMID: 29405996
6
KCNJ12 upregulation via circRNA-miRNA axis promotes bladder cancer progression through EMT and GSK3β/AKT signaling
PMID: 34755307
7
KCNJ12 DNA methylation as novel biomarker for colorectal cancer detection in peripheral blood
PMID: 33892797
Disease Associationsⓘ20
HypercholesterolemiaOpen Targets
0.41Moderate
type 2 diabetes mellitusOpen Targets
0.40Moderate
neurodegenerative diseaseOpen Targets
0.38Weak
health study participationOpen Targets
0.32Weak
Hallux valgusOpen Targets
0.32Weak
hypertensionOpen Targets
0.31Weak
obesityOpen Targets
0.30Weak
Abnormality of the skeletal systemOpen Targets
0.12Weak
eye injuryOpen Targets
0.06Suggestive
Blackfan-Diamond anemiaOpen Targets
0.05Suggestive
primary familial polycythemia due to EPO receptor mutationOpen Targets
0.05Suggestive
alpha thalassemia-intellectual disability syndrome type 1Open Targets
0.05Suggestive
Alpha-thalassemia - intellectual disability syndrome linked to chromosome 16Open Targets
0.05Suggestive
Varicose veinsOpen Targets
0.05Suggestive
vein disorderOpen Targets
0.05Suggestive
hemoglobin D diseaseOpen Targets
0.04Suggestive
hemolytic anemia due to diphosphoglycerate mutase deficiencyOpen Targets
0.04Suggestive
delta-beta-thalassemiaOpen Targets
0.04Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.04Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
LIN7AProtein interaction99%LIN7CProtein interaction98%KCNQ5Protein interaction97%KCNQ2Protein interaction97%KCNJ2Protein interaction95%KCNQ1Protein interaction95%
Tissue Expression6 tissues
Heart
100%
Lung
16%
Brain
15%
Bone Marrow
9%
Ovary
4%
Liver
2%
Gene Interaction Network
Click a node to explore
KCNJ12LIN7ALIN7CKCNQ5KCNQ2KCNJ2KCNQ1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q14500
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.37Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.12 [0.05–0.37]
RankingsWhere KCNJ12 stands among ~20K protein-coding genes
  • #8,966of 20,598
    Most Researched49
  • #1,706of 17,882
    Most Constrained (LOEUF)0.37 · top 10%
Genes detectedKCNJ12
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The human inward rectifying K+ channel Kir 2.2 (KCNJ12) gene: gene structure, assignment to chromosome 17p11.1, and identification of a simple tandem repeat polymorphism.
PMID: 9027495
Genomics · 1997
1.00
2
Experimental Mapping of the Canine KCNJ2 and KCNJ12 Gene Structures and Functional Analysis of the Canine K(IR)2.2 ion Channel.
PMID: 22363290
Front Physiol · 2012
0.90
3
Identification of human Kir2.2 (KCNJ12) gene encoding functional inward rectifier potassium channel in both mammalian cells and Xenopus oocytes.
PMID: 12417321
FEBS Lett · 2002
0.80
4
Whole genome and exome sequencing realignment supports the assignment of KCNJ12, KCNJ17, and KCNJ18 paralogous genes in thyrotoxic periodic paralysis locus: functional characterization of two polymorphic Kir2.6 isoforms.
PMID: 27008341
Mol Genet Genomics · 2016
0.70
5
Whole exome sequencing identifies a KCNJ12 mutation as a cause of familial dilated cardiomyopathy.
PMID: 28816949
Medicine (Baltimore) · 2017
0.60