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Gene

KCNC3

potassium voltage-gated channel subfamily C member 3

Summary

Cytogenetic location
19q13.33
Coordinates
Chromosome 19: 50,311,937–50,329,377 (GRCh38)
Exons
5 exons across 17.4 kb
Protein
757 amino acids
Inheritance
Autosomal dominant
Also known as
Kv3.3
Previous symbols
SCA13
MANE Select transcript
ENST00000477616.2NM_004977.3
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 report relevant to this gene

  • Autosomal Dominant Cerebellar Ataxias (SCAs and DRPLA)

    • Autosomal Dominant Cerebellar AtaxiaAutosomal dominant

      A group of dominantly inherited ataxias, including SCA1, SCA2, SCA3 (Machado-Joseph disease), SCA6, SCA7, SCA17, SCA27B and DRPLA, caused by repeat expansions or point variants in genes such as ATXN1, ATXN2, ATXN3, CACNA1A, ATXN7, TBP, ATN1 and FGF14.

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

      Genetic counselling and presymptomatic testing for relatives, physiotherapy and symptom management; some forms, such as SCA27B, may respond to specific medication.

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 KCNC3 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 19, with a bracket where KCNC3 sits. KCNC3 and 34 genes near it on chromosome 19, from 49,820,657 to 50,820,656.Chromosome 1919q13.33p13.3p13.2p13.13p13.12p13.11p12p11q11q12q13.11q13.12q13.13q13.2q13.31q13.32q13.33q13.41q13.42q13.4350 Mb50.2 Mb50.4 Mb50.6 Mb50.8 MbMED25: 49,818,269–49,840,384, forward strandMED25PTOV1: 49,850,735–49,860,744, forward strandPTOV1PNKP: 49,859,882–49,878,351, reverse strandPNKPAKT1S1: 49,869,023–49,879,470, reverse strandAKT1S1TBC1D17: 49,877,501–49,888,751, forward strandIL4I1: 49,889,654–49,929,926, reverse strandNUP62: 49,906,825–49,929,926, reverse strand: 49,909,501–49,958,391, reverse strandATF5: 49,923,910–49,933,935, forward strandATF5SIGLEC11: 49,948,910–49,961,172, reverse strandSIGLEC11SIGLEC16: 49,969,600–49,975,819, forward strandSIGLEC16VRK3: 49,974,537–50,025,946, reverse strandVRK3ZNF473: 50,025,714–50,053,414, forward strandZNF473IZUMO2: 50,152,537–50,163,281, reverse strandIZUMO2MYH14: 50,188,181–50,310,545, forward strandMYH14KCNC3: 50,311,937–50,329,377, reverse strandKCNC3NR1H2: 50,329,639–50,383,404, forward strandNR1H2NAPSA: 50,358,472–50,369,342, reverse strandNAPSAPOLD1: 50,384,204–50,418,018, forward strandPOLD1: 50,415,799–50,428,409, forward strandSPIB: 50,418,931–50,431,314, forward strandSPIBMYBPC2: 50,432,772–50,466,328, forward strandMYBPC2GARIN5A: 50,466,785–50,476,866, reverse strandGARIN5AEMC10: 50,476,400–50,510,789, forward strandEMC10JOSD2: 50,505,995–50,511,376, reverse strandJOSD2ASPDH: 50,511,568–50,514,696, reverse strandASPDHLRRC4B: 50,516,892–50,568,435, reverse strandLRRC4BSYT3: 50,621,307–50,640,040, reverse strandSYT3C19orf81: 50,648,792–50,659,310, forward strandC19orf81SHANK1: 50,659,255–50,719,802, reverse strandSHANK1CLEC11A: 50,723,283–50,725,719, forward strandCLEC11AGPR32: 50,770,464–50,771,732, forward strandGPR32SMIM47: 50,776,141–50,793,142, reverse strandSMIM47ACP4: 50,790,415–50,795,219, forward strandACP4KLK1: 50,819,146–50,823,787, reverse strandKLK1Diagram of KCNC3: 5 exons on the reverse strand of chromosome 19Exon 2: 3 known disease-causing sitesExon 1: 1 known disease-causing siteExon 5: 50,311,937–50,316,091, 4,155 basesExon 4: 50,320,223–50,320,349, 127 basesExon 3: 50,320,593–50,320,784, 192 basesExon 2: 50,322,975–50,324,082, 1,108 basesExon 1: 50,328,213–50,329,377, 1,165 bases543215′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 6.7 kb of the gene's 17.4 kb; 2,274 of those bases code for the protein.

How a genome test reads KCNC3

4

positions in KCNC3 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 17.4 kb of KCNC3, introns included, not only the 6.7 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.). KCNC3 gene: potassium voltage-gated channel subfamily C member 3. Retrieved 16 October 2026, from https://dantelabs.com/genes/kcnc3

Vancouver

Dante Labs. KCNC3 gene: potassium voltage-gated channel subfamily C member 3 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/kcnc3

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