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Gene

KCNJ10

potassium inwardly rectifying channel subfamily J member 10

Summary

Cytogenetic location
1q23.2
Coordinates
Chromosome 1: 160,037,467–160,070,160 (GRCh38)
Exons
2 exons across 32.7 kb
Protein
379 amino acids
Inheritance
Autosomal dominant Autosomal recessive
Also known as
Kir4.1, Kir1.2
MANE Select transcript
ENST00000644903.1NM_002241.5
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

  • Dystonia, Myotonia, Paroxysmal Dyskinesia

    • Paroxysmal DyskinesiaAutosomal dominant

      Paroxysmal dyskinesia (PD) is a rare heterogenous group of movement disorders manifesting as abnormal involuntary movements that recur episodically and last only a brief time. PD includes paroxysmal kinesigenic dyskinesia (PKD), paroxysmal non-kinesigenic dyskinesia (PNKD), paroxysmal exertion-induced dyskinesia (PED) and a variant form of PKD, infantile convulsion and choreoathetosis (ICCA syndrome).

      • 1 GenCC submitter rates it definitive or strong
    • Inherited Dystonia

      Umbrella term covering inherited dystonias, including DYT-TOR1A early-onset generalized dystonia, DYT-THAP1, myoclonus-dystonia (SGCE) and KMT2B-related dystonia.

      • 1 GenCC submitter rates it definitive or strong

  • Familial Hypomagnesemia

    • EAST/SeSAME SyndromeAutosomal recessive

      A recessive KCNJ10 disorder combining epilepsy, ataxia, sensorineural deafness and a salt- and magnesium-wasting kidney tubulopathy.

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

      Knowing supports coordinated neurological, hearing and electrolyte care.

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.
Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How KCNJ10 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 1, with a bracket where KCNJ10 sits. KCNJ10 and 27 genes near it on chromosome 1, from 159,553,814 to 160,553,813.Chromosome 11q23.2p36.33p36.32p36.31p36.23p36.22p36.21p36.13p36.12p36.11p35.3p35.2p35.1p34.3p34.2p34.1p33p32.3p32.2p32.1p31.3p31.2p31.1p22.3p22.2p22.1p21.3p21.2p21.1p13.3p13.2p13.1p12p11.2p11.1q11q12q21.1q21.2q21.3q22q23.1q23.2q23.3q24.1q24.2q24.3q25.1q25.2q25.3q31.1q31.2q31.3q32.1q32.2q32.3q41q42.11q42.12q42.13q42.2q42.3q43q44159.6 Mb159.8 Mb160 Mb160.2 Mb160.4 MbAPCS: 159,587,818–159,602,430, forward strandAPCSCRP: 159,711,438–159,714,595, reverse strandCRPDUSP23: 159,780,931–159,782,543, forward strandDUSP23FCRL6: 159,800,503–159,816,262, forward strandFCRL6SLAMF8: 159,826,682–159,837,492, forward strandSLAMF8: 159,834,474–159,873,053, reverse strandVSIG8: 159,854,316–159,862,657, reverse strandVSIG8CFAP45: 159,872,364–159,900,165, reverse strandCFAP45TAGLN2: 159,918,106–159,925,546, reverse strandTAGLN2IGSF9: 159,927,039–159,949,468, reverse strandIGSF9SLAMF9: 159,951,492–159,954,237, reverse strandSLAMF9PIGM: 160,024,953–160,031,990, reverse strandPIGMKCNJ10: 160,037,467–160,070,160, reverse strandKCNJ10KCNJ9: 160,081,501–160,094,277, forward strandKCNJ9IGSF8: 160,091,340–160,101,109, reverse strandIGSF8ATP1A2: 160,115,755–160,143,605, forward strandATP1A2ATP1A4: 160,151,586–160,187,593, forward strandATP1A4CASQ1: 160,190,212–160,201,886, forward strandCASQ1PEA15: 160,205,328–160,220,335, forward strandPEA15DCAF8: 160,214,840–160,263,388, reverse strandDCAF8: 160,216,800–160,285,130, reverse strandPEX19: 160,266,229–160,286,348, reverse strandPEX19COPA: 160,288,580–160,343,566, reverse strandCOPANCSTN: 160,343,294–160,358,957, forward strandNCSTNNHLH1: 160,367,071–160,372,846, forward strandNHLH1VANGL2: 160,400,528–160,428,678, forward strandVANGL2SLAMF6: 160,484,817–160,523,273, reverse strandSLAMF6CD84: 160,541,095–160,579,544, reverse strandCD84Diagram of KCNJ10: 2 exons on the reverse strand of chromosome 1Exon 2: 23 known disease-causing sitesExon 2: 160,037,467–160,042,532, 5,066 basesExon 1: 160,070,022–160,070,160, 139 bases215′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 5.2 kb of the gene's 32.7 kb; 1,140 of those bases code for the protein.

How a genome test reads KCNJ10

24

positions in KCNJ10 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 32.7 kb of KCNJ10, introns included, not only the 5.2 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.). KCNJ10 gene: potassium inwardly rectifying channel subfamily J member 10. Retrieved 16 October 2026, from https://dantelabs.com/genes/kcnj10

Vancouver

Dante Labs. KCNJ10 gene: potassium inwardly rectifying channel subfamily J member 10 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/kcnj10

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