Gene
PRODH
proline dehydrogenase 1
- HGNC 9453↗(opens in a new tab)
- Ensembl gene ENSG00000100033↗(opens in a new tab)
- NCBI Gene 5625↗(opens in a new tab)
Summary
- Cytogenetic location
- 22q11.21
- Coordinates
- Chromosome 22: 18,912,781–18,936,293 (GRCh38)
- Exons
- 14 exons across 23.5 kb
- Protein
- 600 amino acids
- Also known as
- HSPOX2, PRODH1, PIG6, PRODH2, TP53I6, POX
- MANE Select transcript
- ENST00000357068.11NM_016335.6
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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No Genome Test report reads PRODH yet. The test sequences your whole genome, so your data includes it all the same.
How PRODH is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Known disease-causing sites
- Stretches short reads find hard
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 2.2 kb of the gene's 23.5 kb; 1,803 of those bases code for the protein.
How a genome test reads PRODH
22
positions in PRODH where ClinVar classes a change as pathogenic or likely pathogenic
22 of them (100%) fall in regions short reads find hard.
- Low mappability (22 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.
- Segmental duplications (22 sites)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.
A whole genome covers all 23.5 kb of PRODH, introns included, not only the 2.2 kb of exons.
Traits linked to PRODH
The Human Phenotype Ontology lists these as features of the rare conditions PRODH can cause. Each trait's page lists the other genes linked to it.
External links
Gene records
Transcript
Protein
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.
- 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
Dante Labs. (n.d.). PRODH gene: proline dehydrogenase 1. Retrieved 16 October 2026, from https://dantelabs.com/genes/prodh
Dante Labs. PRODH gene: proline dehydrogenase 1 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/prodh
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