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Gene

ZFHX3

zinc finger homeobox 3

Summary

Cytogenetic location
16q22.2-q22.3
Coordinates
Chromosome 16: 72,782,885–73,048,128 (GRCh38)
Exons
10 exons across 265.2 kb
Protein
3,703 amino acids
Inheritance
Autosomal dominant
Also known as
ZNF927, FLJ26184
Previous symbols
ATBF1, C16orf47
MANE Select transcript
ENST00000268489.10NM_006885.4
Locus type
gene with protein product

Reference genomeGRCh38

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

Curated reports 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.

      • 1 GenCC submitter rates 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.

Broader groupings

  • Epilepsy (Full Panel)

    • Epilepsy

      One of 171 genes linked to it

      A brain disorder characterized by episodes of abnormally increased neuronal discharge resulting in transient episodes of sensory or motor neurological dysfunction, or psychic dysfunction. These episodes may or may not be associated with loss of consciousness or convulsions.

      • 1 GenCC submitter rates it definitive or strong

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 ZFHX3 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 16, with a bracket where ZFHX3 sits. ZFHX3 and 0 genes near it on chromosome 16, from 72,415,507 to 73,415,506.Chromosome 1616q22.2-q22.3p13.3p13.2p13.13p13.12p13.11p12.3p12.2p12.1p11.2p11.1q11.1q11.2q12.1q12.2q13q21q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.372.6 Mb72.8 Mb73 Mb73.2 Mb73.4 MbZFHX3: 72,782,885–73,048,128, reverse strandZFHX3Diagram of ZFHX3: 10 exons on the reverse strand of chromosome 16Exon 9: 1 known disease-causing siteExon 2: 1 known disease-causing siteExon 10: 72,782,885–72,788,848, 5,964 basesExon 9: 72,793,255–72,798,714, 5,460 basesExon 8: 72,800,027–72,800,129, 103 basesExon 7: 72,811,577–72,811,777, 201 basesExon 6: 72,811,905–72,812,038, 134 basesExon 5: 72,829,779–72,829,859, 81 basesExon 4: 72,889,731–72,889,962, 232 basesExon 3: 72,950,469–72,950,965, 497 basesExon 2: 72,957,427–72,960,194, 2,768 basesExon 1: 73,047,752–73,048,128, 377 bases10864215′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 15.8 kb of the gene's 265.2 kb; 11,112 of those bases code for the protein.

How a genome test reads ZFHX3

2

positions in ZFHX3 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 265.2 kb of ZFHX3, introns included, not only the 15.8 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.). ZFHX3 gene: zinc finger homeobox 3. Retrieved 16 October 2026, from https://dantelabs.com/genes/zfhx3

Vancouver

Dante Labs. ZFHX3 gene: zinc finger homeobox 3 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/zfhx3

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