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Gene

KCNJ11

potassium inwardly rectifying channel subfamily J member 11

Summary

Cytogenetic location
11p15.1
Coordinates
Chromosome 11: 17,385,248–17,388,659 (GRCh38)
Exons
1 exon across 3.4 kb
Protein
390 amino acids
Inheritance
Autosomal dominant
Also known as
Kir6.2, BIR
MANE Select transcript
ENST00000339994.5NM_000525.4
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Diabetes Mellitus

    • A metabolic disorder characterized by abnormally high blood sugar levels due to diminished production of insulin or insulin resistance/desensitization.

      • ClinGen rates the link definitive
      • 6 GenCC submitters rate it definitive or strong
      What knowing changes

      Teplizumab (Tzield) for stage 2 presymptomatic T1D (FDA approved 2022); insulin replacement at clinical T1D onset; TrialNet autoantibody screening for high-risk relatives; closed-loop insulin delivery systems

  • Hyperinsulinism

    • Islet Cell AdenomatosisAutosomal dominant

      A sporadic or inherited disorder characterized by the focal or diffuse proliferation of the cells of the islets of Langerhans in the pancreas. It results in hyperinsulinemia and hypoglycemia.

      • ClinGen rates the link definitive
      • 8 GenCC submitters rate it definitive or strong

    • Abnormally high levels of insulin in the blood.

      • ClinGen rates the link definitive
      • 8 GenCC submitters rate it definitive or strong

  • Maturity-Onset Diabetes Of The Young

    • Autosomal dominant

      MODY (maturity-onset diabetes of the young) is a rare, familial, clinically and genetically heterogeneous form of diabetes characterized by young age of onset (generally 10-45 years of age) with maintenance of endogenous insulin production, lack of pancreatic beta-cell autoimmunity, absence of obesity and insulin resistance and extra-pancreatic manifestations in some subtypes.

      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

      GCK-MODY: no treatment needed; HNF1A/HNF4A-MODY: low-dose sulfonylureas; HNF1B-MODY: insulin + renal monitoring; T2D polygenic: lifestyle modification, metformin, GLP-1 agonists, SGLT2 inhibitors based on comorbidity profile

  • Neonatal Diabetes Mellitus

    • Diabetes diagnosed in the first months of life, either permanent or transient, caused by single-gene defects in insulin production or secretion.

      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

      Most people with KCNJ11 or ABCC8 neonatal diabetes can switch from insulin injections to sulfonylurea tablets, often with better glucose control.

Inheritance

Autosomal dominant
One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.

How KCNJ11 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
Chromosome 11, with a bracket where KCNJ11 sits. KCNJ11 and 11 genes near it on chromosome 11, from 16,886,954 to 17,886,953.Chromosome 1111p15.1p15.5p15.4p15.3p15.2p15.1p14.3p14.2p14.1p13p12p11.2p11.12p11.11q11q12.1q12.2q12.3q13.1q13.2q13.3q13.4q13.5q14.1q14.2q14.3q21q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.3q2517 Mb17.2 Mb17.4 Mb17.6 Mb17.8 MbPLEKHA7: 16,777,297–17,014,589, reverse strandPLEKHA7RPS13: 17,066,750–17,077,729, reverse strandRPS13PIK3C2A: 17,077,730–17,207,986, reverse strandPIK3C2ANUCB2: 17,208,072–17,349,980, forward strandNUCB2NCR3LG1: 17,351,677–17,377,341, forward strandNCR3LG1KCNJ11: 17,385,248–17,388,659, reverse strandKCNJ11ABCC8: 17,392,498–17,476,894, reverse strandABCC8USH1C: 17,493,895–17,544,435, reverse strandUSH1COTOG: 17,547,259–17,647,150, forward strandOTOGMYOD1: 17,719,565–17,722,136, forward strandMYOD1KCNC1: 17,734,724–17,856,811, forward strandKCNC1SERGEF: 17,788,043–18,013,199, reverse strandSERGEFDiagram of KCNJ11: one exon on the reverse strand of chromosome 11Exon 1: 63 known disease-causing sitesExon 1: 17,385,248–17,388,659, 3,412 bases15′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 3.4 kb of the gene's 3.4 kb; 1,173 of those bases code for the protein.

How a genome test reads KCNJ11

63

positions in KCNJ11 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 3.4 kb of KCNJ11, introns included, not only the 3.4 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). KCNJ11 gene: potassium inwardly rectifying channel subfamily J member 11. Retrieved 17 October 2026, from https://dantelabs.com/genes/kcnj11

Vancouver

Dante Labs. KCNJ11 gene: potassium inwardly rectifying channel subfamily J member 11 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/kcnj11

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