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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KCNRG
potassium channel regulator
Chromosome 13 · 13q14.2
NCBI Gene: 283518Ensembl: ENSG00000198553.9HGNC: HGNC:18893UniProt: Q8N5I3
19PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingidentical protein bindingnegative regulation of delayed rectifier potassium channel activityendoplasmic reticulumatrial fibrillationAbnormality of the skeletal systemeye injuryendometriosis
✦AI Summary

KCNRG (potassium channel regulator) is a tumor suppressor gene located at chromosome 13.3 that functions as a negative regulator of potassium channel activity 1. The protein suppresses K+ channel activity, particularly targeting Kv1 family channels by retaining them in endomembranes and reducing their surface expression 12. KCNRG exhibits pro-apoptotic and antiproliferative effects in various cell lines, consistent with its role as a tumor suppressor 3. Mechanistically, KCNRG associates with the N-terminal T1 domain of potassium channels and functions as an ER-associated protein to regulate channel trafficking 2. The gene is clinically significant across multiple disease contexts: deletions and mutations are frequent in B-cell chr13 lymphocytic leukemia, multiple myeloma, and hepatocellular carcinoma, where haploinsufficiency contributes to tumor progression 34. Additionally, KCNRG serves as an autoantigen in autoimmune conditions, with anti-KCNRG antibodies strongly associated with pulmonary manifestations in autoimmune polyendocrine syndrome type 1 (APS-1) and thymoma-associated pneumonitis 567. The protein's expression is predominantly restricted to bronchial epithelial cells, making it a specific target in pulmonary autoimmunity 5.

Sources cited
1
KCNRG is located at 13q14.3 and suppresses K+ channel activity in prostate cells
PMID: 12650944
2
KCNRG regulates Kv1 family channels by retaining them in endomembranes and is ER-associated
PMID: 19968958
3
KCNRG shows pro-apoptotic and antiproliferative effects and is frequently deleted in CLL and MM
PMID: 20237900
4
KCNRG mutations and allelic loss are associated with hepatocellular carcinoma progression
PMID: 16819283
5
KCNRG serves as an autoantigen in APS-1 pulmonary manifestations and is expressed in bronchial epithelium
PMID: 19251657
6
Anti-KCNRG antibodies are associated with pneumonitis in APECED patients
PMID: 31167928
7
KCNRG autoantibodies are present in thymoma-associated pneumonitis
PMID: 38954150
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.28Weak
Abnormality of the skeletal systemOpen Targets
0.27Weak
eye injuryOpen Targets
0.22Weak
endometriosisOpen Targets
0.17Weak
hypothyroidismOpen Targets
0.15Weak
clonal hematopoiesisOpen Targets
0.13Weak
disease of peritoneumOpen Targets
0.12Weak
smoking initiationOpen Targets
0.12Weak
prostate cancerOpen Targets
0.12Weak
liver diseaseOpen Targets
0.11Weak
multiple sclerosisOpen Targets
0.10Weak
psoriasisOpen Targets
0.10Weak
temporomandibular joint disorderOpen Targets
0.10Suggestive
primary biliary cirrhosisOpen Targets
0.10Suggestive
agingOpen Targets
0.09Suggestive
uterine fibroidOpen Targets
0.09Suggestive
autoimmune diseaseOpen Targets
0.09Suggestive
lymphatic system diseaseOpen Targets
0.09Suggestive
Eczematoid dermatitisOpen Targets
0.07Suggestive
Hashimoto's thyroiditisOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TRIM13Protein interaction94%FAUProtein interaction91%KCTD3Shared pathway50%IKZF4Shared pathway50%KCTD14Shared pathway50%KCTD15Shared pathway50%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
50%
Heart
28%
Ovary
26%
Brain
24%
Liver
22%
Gene Interaction Network
Click a node to explore
KCNRGTRIM13FAUKCTD3IKZF4KCTD14KCTD15
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8N5I3
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.50LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.01 [0.70–1.50]
RankingsWhere KCNRG stands among ~20K protein-coding genes
  • #14,451of 20,598
    Most Researched19
  • #15,154of 17,882
    Most Constrained (LOEUF)1.50
Genes detectedKCNRG
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A new human gene KCNRG encoding potassium channel regulating protein is a cancer suppressor gene candidate located in 13q14.3.
PMID: 12650944
FEBS Lett · 2003
1.00
2
Lymphocyte-Directed Immunomodulation Remits Thymoma-Associated Autoimmune Pneumonitis.
PMID: 38954150
J Clin Immunol · 2024
0.90
3
Pro-apoptotic and antiproliferative activity of human KCNRG, a putative tumor suppressor in 13q14 region.
PMID: 20237900
Tumour Biol · 2010
0.80
4
Genetic and expression analysis of the KCNRG gene in hepatocellular carcinomas.
PMID: 16819283
Exp Mol Med · 2006
0.70
5
Pulmonary autoimmunity as a feature of autoimmune polyendocrine syndrome type 1 and identification of KCNRG as a bronchial autoantigen.
PMID: 19251657
Proc Natl Acad Sci U S A · 2009
0.60