Proline oxidase

PRODH
Identifiers
Aliases	PRODH, HSPOX2, PIG6, POX, PRODH1, PRODH2, TP53I6, Proline oxidase, proline dehydrogenase 1
External IDs	OMIM: 606810; MGI: 97770; HomoloGene: 40764; GeneCards: PRODH; OMA:PRODH - orthologs
Gene location (Human)
Chr.	Chromosome 22 (human)
End	18,936,553 bp
Gene location (Mouse)
Chr.	Chromosome 16 (mouse)
End	17,908,067 bp
RNA expression pattern
	Top expressed in
	skin of leg; ; skin of abdomen; ; tibial nerve; ; Amygdala; ; caudate nucleus; ; right lung; ; nucleus accumbens; ; putamen; ; apex of heart; ; sural nerve;
	Top expressed in
	human kidney; ; right kidney; ; renal cortex; ; proximal tubule; ; hepatobiliary system; ; liver; ; muscle of thigh; ; primary visual cortex; ; cerebellar cortex; ; superior frontal gyrus;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	oxidoreductase activity; FAD binding; proline dehydrogenase activity;
Cellular component	mitochondrial inner membrane; mitochondrial matrix; mitochondrion;
Biological process	intrinsic apoptotic signaling pathway in response to oxidative stress; positive regulation of cell death; 4-hydroxyproline catabolic process; proline catabolic process; proline metabolic process; proline catabolic process to glutamate;
	Sources:Amigo / QuickGO
Orthologs
	5625
	19125
	ENSG00000100033
	ENSMUSG00000003526
	O43272
	Q9WU79
	NM_001195226; NM_016335
	NM_011172
	NP_001182155; NP_057419
	NP_035302
	Wikidata
View/Edit Human	View/Edit Mouse

Proline dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the PRODH gene.

The protein encoded by this gene is a mitochondrial proline dehydrogenase which catalyzes the first step in proline catabolism. Deletion of this gene has been associated with type I hyperprolinemia. The gene is located on chromosome 22q11.21, a region which has also been associated with the contiguous gene deletion syndromes: DiGeorge syndrome and CATCH22 syndrome.