Homo sapiens Protein: PRODH
Summary
InnateDB Protein IDBP-1201.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PRODH
Protein Name proline dehydrogenase (oxidase) 1
Synonyms HSPOX2; PIG6; POX; PRODH1; PRODH2; TP53I6;
Species Homo sapiens
Ensembl Protein ENSP00000349577
InnateDB Gene IDBG-1197 (PRODH)
Protein Structure
UniProt Annotation
Function Converts proline to delta-1-pyrroline-5-carboxylate.
Subcellular Localization Mitochondrion matrix.
Disease Associations Hyperprolinemia 1 (HP-1) [MIM:239500]: Characterized by elevated serum proline levels. May be involved in the psychiatric and behavioral phenotypes associated with the 22q11 velocardiofacial and DiGeorge syndrome. {ECO:0000269PubMed:12217952}. Note=The disease is caused by mutations affecting the gene represented in this entry.Schizophrenia 4 (SCZD4) [MIM:600850]: A complex, multifactorial psychotic disorder or group of disorders characterized by disturbances in the form and content of thought (e.g. delusions, hallucinations), in mood (e.g. inappropriate affect), in sense of self and relationship to the external world (e.g. loss of ego boundaries, withdrawal), and in behavior (e.g bizarre or apparently purposeless behavior). Although it affects emotions, it is distinguished from mood disorders in which such disturbances are primary. Similarly, there may be mild impairment of cognitive function, and it is distinguished from the dementias in which disturbed cognitive function is considered primary. Some patients manifest schizophrenic as well as bipolar disorder symptoms and are often given the diagnosis of schizoaffective disorder. {ECO:0000269PubMed:11891283}. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry.
Tissue Specificity Expressed in lung, skeletal muscle and brain, to a lesser extent in heart and kidney, and weakly in liver, placenta and pancreas.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
Gene Ontology

Molecular Function
Accession GO Term
GO:0004657 proline dehydrogenase activity
GO:0071949 FAD binding
Biological Process
GO:0006537 glutamate biosynthetic process
GO:0006560 proline metabolic process
GO:0006562 proline catabolic process
GO:0008631 intrinsic apoptotic signaling pathway in response to oxidative stress
GO:0010133 proline catabolic process to glutamate
GO:0019470 4-hydroxyproline catabolic process
GO:0034641 cellular nitrogen compound metabolic process
GO:0044281 small molecule metabolic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
Protein Structure and Domains
PDB ID
InterPro IPR002872 Proline dehydrogenase domain
IPR029041 FAD-linked oxidoreductase-like
PFAM PF01619
PRINTS
PIRSF
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt O43272
PhosphoSite PhosphoSite-O43272
TrEMBL E7EQL6
UniProt Splice Variant
Entrez Gene 5625
UniGene Hs.517352
RefSeq
HUGO HGNC:9453
OMIM 606810
CCDS CCDS13754
HPRD 08433
IMGT
EMBL AB209472 AC007326 AF010310 AF120278 BC068260 BC094736 BC118597 BC121809 U79754 U82381
GenPept AAB88789 AAC39529 AAD24775 AAF21464 AAH68260 AAH94736 AAI18598 AAI21810 BAD92709