Homo sapiens Gene: PRODH
Summary
InnateDB Gene IDBG-1197.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PRODH
Gene Name proline dehydrogenase (oxidase) 1
Synonyms HSPOX2; PIG6; POX; PRODH1; PRODH2; TP53I6
Species Homo sapiens
Ensembl Gene ENSG00000100033
Encoded Proteins
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
proline dehydrogenase (oxidase) 1
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene encodes a mitochondrial protein that catalyzes the first step in proline degradation. Mutations in this gene are associated with hyperprolinemia type 1 and susceptibility to schizophrenia 4 (SCZD4). This gene is located on chromosome 22q11.21, a region which has also been associated with the contiguous gene deletion syndromes, DiGeorge and CATCH22. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2010]
Gene Information
Type Protein coding
Genomic Location Chromosome 22:18912777-18936553
Strand Reverse strand
Band q11.21
Transcripts
ENST00000334029 ENSP00000334726
ENST00000357068 ENSP00000349577
ENST00000313755
ENST00000399694
ENST00000420436 ENSP00000410805
ENST00000429300
ENST00000446371
ENST00000438924 ENSP00000409742
ENST00000450579 ENSP00000396806
ENST00000457083 ENSP00000413941
ENST00000491604
ENST00000482858
ENST00000496625
ENST00000609229
ENST00000610940 ENSP00000480347
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
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Not yet available
Pathways
NETPATH
REACTOME
Proline catabolism pathway
Metabolism of amino acids and derivatives pathway
Metabolism pathway
KEGG
Arginine and proline metabolism pathway
INOH
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.517352
RefSeq NM_001195226 NM_016335
HUGO
OMIM
CCDS CCDS13754 CCDS56223
HPRD 08433
IMGT
EMBL
GenPept
RNA Seq Atlas