Hydroxyproline dehydrogenase

PRODH2
Identifiers
Aliases	PRODH2, HSPOX1, HYPDH, proline dehydrogenase 2
External IDs	OMIM: 616377; MGI: 1929093; GeneCards: PRODH2
Enzyme activity
Gene location (Human)
Chr.	Chromosome 19 (human)
End	35,813,299 bp
Gene location (Mouse)
Chr.	Chromosome 7 (mouse)
End	30,212,827 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; kidney tubule; ; glomerulus; ; metanephric glomerulus; ; human kidney; ; Skeletal muscle tissue of rectus abdominis; ; renal medulla; ; sperm; ; body of pancreas; ; cartilage tissue;
	Top expressed in
	left lobe of liver; ; right kidney; ; yolk sac; ; human kidney; ; gallbladder; ; parietal layer of glomerular capsule; ; urethra; ; tongue; ; morula; ; embryo;
	More reference expression data
	n/a
Gene ontology
Molecular function	oxidoreductase activity; FAD binding; oxidoreductase activity, acting on the CH-NH group of donors; proline dehydrogenase activity;
Cellular component	mitochondrion; mitochondrial inner membrane;
Biological process	proline metabolic process; proline catabolic process; proline catabolic process to glutamate; glyoxylate metabolic process;
	Sources:Amigo / QuickGO
Orthologs
Databases	NCBI: entry; OMA: entry
	58510
	56189
	ENSG00000250799
	ENSMUSG00000036892
	Q9UF12
	Q8VCZ9
	NM_021232; NM_001378292; NM_001378293; NM_001378294
	NM_019546
	NP_067055; NP_001365221; NP_001365222; NP_001365223
	NP_062419
	Wikidata
View/Edit Human	View/Edit Mouse

Hydroxyproline dehydrogenase, also called proline dehydrogenase 2, is an enzyme that in humans is encoded by the PRODH2 gene. This enzyme is located in the mitochondrial inner membrane, where it is involved in breaking down hydroxyproline, a modified form of the amino acid proline and major component of collagen. The human enzyme is also able to act on proline (similar to PRODH), but with much lower affinity.

Function

As an enzyme, hydroxyproline dehydrogenase (EC 1.5.5.3) acts on hydroxyproline to remove the two hydrogens bonded to the nitrogen atom. More precisely, it catalyses the following reaction:

trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H⁺

In humans, the quinone cofactor used is coenzyme Q₁₀, and it does not act on other diastereomers like trans-4-D-hydroxyproline or cis-4-L-hydroxyproline. It is also able to act on proline as a proline dehydrogenase (EC 1.5.5.3) with low affinity.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000250799 – Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000036892 – Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
1 2 3 4 5 "Hydroxyproline dehydrogenase". uniprot.org. UniProt consortium. Retrieved 2026-10-16.
↑ Chalecka M, Hermanowicz JM, Palka J, Surazynski A (2025-05-16). "The new insight into the role of hydroxyproline in metabolism of cancer cells". Frontiers in Cell and Developmental Biology. 13. doi:10.3389/fcell.2025.1556770.
↑ EC 1.5.5.3

This article on a gene on human chromosome 19 is a stub. You can help Wikipedia by adding missing information.

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000250799 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000036892 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[uniprot-5] 1 2 3 4 5 "Hydroxyproline dehydrogenase". uniprot.org. UniProt consortium. Retrieved 2026-10-16.

[Chalecka2025-6] Chalecka M, Hermanowicz JM, Palka J, Surazynski A (2025-05-16). "The new insight into the role of hydroxyproline in metabolism of cancer cells". Frontiers in Cell and Developmental Biology. 13. doi:10.3389/fcell.2025.1556770.

[7] EC 1.5.5.3