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Gene

HADH

hydroxyacyl-CoA dehydrogenase

Summary

Cytogenetic location
4q25
Coordinates
Chromosome 4: 107,989,889–108,035,171 (GRCh38)
Exons
8 exons across 45.3 kb
Protein
314 amino acids
Inheritance
Autosomal dominant Autosomal recessive
Also known as
HADH1, SCHAD
Previous symbols
HADHSC
MANE Select transcript
ENST00000309522.8NM_005327.7
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

  • Fatty Acid Oxidation & Carnitine Cycle Disorders

    • Disorders of Fatty Acid Oxidation and KetogenesisAutosomal recessive

      A group of enzyme and transporter defects, including MCAD, VLCAD, LCHAD, SCAD and HADH deficiency, primary carnitine deficiency and carnitine-acylcarnitine translocase deficiency, that can cause low blood sugar, muscle breakdown, heart and liver problems during periods of fasting or illness.

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

      Knowing allows avoidance of prolonged fasting, an emergency plan for illness and, where relevant, carnitine or dietary fat modification, which can prevent life-threatening episodes.

  • 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
      • 3 GenCC submitters rate it definitive or strong

    • Abnormally high levels of insulin in the blood.

      • ClinGen rates the link definitive
      • 3 GenCC submitters rate 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.
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 HADH is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 4, with a bracket where HADH sits. HADH and 4 genes near it on chromosome 4, from 107,512,530 to 108,512,529.Chromosome 44q25p16.3p16.2p16.1p15.33p15.32p15.31p15.2p15.1p14p13p12p11q11q12q13.1q13.2q13.3q21.1q21.21q21.22q21.23q21.3q22.1q22.2q22.3q23q24q25q26q27q28.1q28.2q28.3q31.1q31.21q31.22q31.23q31.3q32.1q32.2q32.3q33q34.1q34.2q34.3q35.1q35.2107.6 Mb107.8 Mb108 Mb108.2 Mb108.4 MbPAPSS1: 107,590,276–107,720,395, reverse strandPAPSS1SGMS2: 107,824,563–107,915,047, forward strandSGMS2CYP2U1: 107,931,324–107,953,465, forward strandCYP2U1HADH: 107,989,889–108,035,171, forward strandHADHLEF1: 108,045,592–108,169,198, reverse strandLEF1Diagram of HADH: 8 exons on the forward strand of chromosome 4Exon 1: 3 known disease-causing sitesExon 2: 2 known disease-causing sitesExon 3: 6 known disease-causing sitesExon 4: 4 known disease-causing sitesExon 5: 1 known disease-causing siteExon 6: 4 known disease-causing sitesExon 7: 1 known disease-causing siteExon 8: 3 known disease-causing sitesExon 1: 107,989,889–107,990,064, 176 basesExon 2: 108,009,759–108,009,887, 129 basesExon 3: 108,014,431–108,014,588, 158 basesExon 4: 108,019,540–108,019,666, 127 basesExon 5: 108,023,474–108,023,563, 90 basesExon 6: 108,027,688–108,027,760, 73 basesExon 7: 108,033,176–108,033,292, 117 basesExon 8: 108,034,239–108,035,171, 933 bases123456785′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 1.8 kb of the gene's 45.3 kb; 945 of those bases code for the protein.

How a genome test reads HADH

34

positions in HADH 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 45.3 kb of HADH, introns included, not only the 1.8 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.). HADH gene: hydroxyacyl-CoA dehydrogenase. Retrieved 17 October 2026, from https://dantelabs.com/genes/hadh

Vancouver

Dante Labs. HADH gene: hydroxyacyl-CoA dehydrogenase [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/hadh

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