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Aliases for PRODH Gene

Aliases for PRODH Gene

  • Proline Dehydrogenase (Oxidase) 1 2 3
  • P53-Induced Gene 6 Protein 3 4
  • Proline Oxidase 2 3 4
  • PRODH2 3 6
  • PIG6 3 4
  • Proline Dehydrogenase (Proline Oxidase ) 2
  • Proline Dehydrogenase 1, Mitochondrial 3
  • Tumor Protein P53 Inducible Protein 6 3
  • Proline Oxidase, Mitochondrial 3
  • Proline Oxidase 4
  • EC 63
  • EC 4
  • HSPOX2 3
  • PRODH1 3
  • TP53I6 3
  • SCZD4 6
  • POX2 4
  • POX 3

External Ids for PRODH Gene

Previous GeneCards Identifiers for PRODH Gene

  • GC22M015840
  • GC22M017274
  • GC22M002522

Summaries for PRODH Gene

Entrez Gene Summary for PRODH Gene

  • 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]

GeneCards Summary for PRODH Gene

PRODH (Proline Dehydrogenase (Oxidase) 1) is a Protein Coding gene. Diseases associated with PRODH include hyperprolinemia, type i and schizophrenia 4. Among its related pathways are Cell junction organization and Metabolism. GO annotations related to this gene include FAD binding and proline dehydrogenase activity. An important paralog of this gene is PRODH2.

UniProtKB/Swiss-Prot for PRODH Gene

  • Converts proline to delta-1-pyrroline-5-carboxylate

Gene Wiki entry for PRODH Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for PRODH Gene

Genomics for PRODH Gene

Regulatory Elements for PRODH Gene

Genomic Location for PRODH Gene

18,912,774 bp from pter
18,936,553 bp from pter
23,780 bases
Minus strand

Genomic View for PRODH Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for PRODH Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for PRODH Gene

Proteins for PRODH Gene

  • Protein details for PRODH Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Proline dehydrogenase 1, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • A6NF53
    • O14680
    • Q0P507
    • Q147W8
    • Q504W1
    • Q59FI8
    • Q6NV86
    • Q9UF13

    Protein attributes for PRODH Gene

    600 amino acids
    Molecular mass:
    68002 Da
    Name=FAD; Xref=ChEBI:CHEBI:57692;
    Quaternary structure:
    No Data Available
    • Sequence=AAC39529.1; Type=Frameshift; Positions=540, 563, 568, 577, 580, 582; Evidence={ECO:0000305}; Sequence=AAH68260.1; Type=Frameshift; Positions=123; Evidence={ECO:0000305}; Sequence=AAH94736.1; Type=Frameshift; Positions=40; Evidence={ECO:0000305}; Sequence=AAH94736.1; Type=Miscellaneous discrepancy; Note=Artifact. Missing internal sequence that doesnt correspond to an exon-intron boundary.; Evidence={ECO:0000305}; Sequence=BAD92709.1; Type=Miscellaneous discrepancy; Note=Intron retention. Includes intronic sequence at the 5 end.; Evidence={ECO:0000305};

    Alternative splice isoforms for PRODH Gene


neXtProt entry for PRODH Gene

Proteomics data for PRODH Gene at MOPED

Selected DME Specific Peptides for PRODH Gene

Post-translational modifications for PRODH Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for PRODH Gene

Antibody Products

Domains for PRODH Gene

Protein Domains for PRODH Gene

Suggested Antigen Peptide Sequences for PRODH Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the proline oxidase family.
  • Belongs to the proline oxidase family.
genes like me logo Genes that share domains with PRODH: view

No data available for Gene Families for PRODH Gene

Function for PRODH Gene

Molecular function for PRODH Gene

GENATLAS Biochemistry:
proline dehydrogenase,Drosophila sluggish-A homolog,predominantly expressed in lung,skeletal muscle,brain,heart,kidney,proline metabolic pathway,mitochondrial matrix,involved putatively in any psychiatric disorders
UniProtKB/Swiss-Prot CatalyticActivity:
L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol.
UniProtKB/Swiss-Prot Function:
Converts proline to delta-1-pyrroline-5-carboxylate
UniProtKB/Swiss-Prot Induction:
During p53/TP53-induced apoptosis.

Enzyme Numbers (IUBMB) for PRODH Gene

Gene Ontology (GO) - Molecular Function for PRODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004657 proline dehydrogenase activity IDA 15662599
GO:0071949 FAD binding IDA 15662599
genes like me logo Genes that share ontologies with PRODH: view
genes like me logo Genes that share phenotypes with PRODH: view

Animal Model Products

miRNA for PRODH Gene

miRTarBase miRNAs that target PRODH

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for PRODH

In Situ Assay Products

Flow Cytometry Products

No data available for Animal Models , Transcription Factor Targets and HOMER Transcription for PRODH Gene

Localization for PRODH Gene

Subcellular locations from UniProtKB/Swiss-Prot for PRODH Gene

Mitochondrion matrix.

Subcellular locations from

Jensen Localization Image for PRODH Gene COMPARTMENTS Subcellular localization image for PRODH gene
Compartment Confidence
mitochondrion 5
cytosol 2
extracellular 2
chloroplast 1
peroxisome 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for PRODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005743 mitochondrial inner membrane TAS --
GO:0005759 mitochondrial matrix IEA --
genes like me logo Genes that share ontologies with PRODH: view

Pathways for PRODH Gene

genes like me logo Genes that share pathways with PRODH: view

Pathways by source for PRODH Gene

PCR Array Products

UniProtKB/Swiss-Prot O43272-PROD_HUMAN

  • Pathway: Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 1/2

Gene Ontology (GO) - Biological Process for PRODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006560 proline metabolic process TAS 9385373
GO:0006562 proline catabolic process TAS --
GO:0008631 intrinsic apoptotic signaling pathway in response to oxidative stress NAS 9305847
GO:0010133 proline catabolic process to glutamate IEA --
GO:0019470 4-hydroxyproline catabolic process TAS 21998747
genes like me logo Genes that share ontologies with PRODH: view

Drugs for PRODH Gene

(1) Drugbank Compounds for PRODH Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
  • (-)-(S)-Proline
147-85-3 145742 Target, Enzyme substrate approved, nutraceutical

(10) HMDB Compounds for PRODH Gene

Compound Synonyms Cas Number PubMed IDs
1-Pyrroline-5-carboxylic acid
  • (S)-1-pyrroline-5-carboxylate
4-Hydroxy-L-glutamic acid
  • (2S)-2-amino-4-hydroxypentanedioate
  • (2S)-4-hydroxypyrrolidine-2-carboxylic acid
  • 1H-Purin-6-amine flavin dinucleotide
Hydrogen Ion
  • Hydrogen cation

(6) Novoseek inferred chemical compound relationships for PRODH Gene

Compound -log(P) Hits PubMed IDs
delta(1)pyrroline-5-carboxylate 93.7 9
proline 87.2 68
hydroxyproline 48 1
abscisic acid 37.4 1
glutamate 37 3
genes like me logo Genes that share compounds with PRODH: view

Transcripts for PRODH Gene

Unigene Clusters for PRODH Gene

Proline dehydrogenase (oxidase) 1:
Representative Sequences:

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for PRODH

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for PRODH Gene

ExUns: 1 ^ 2a · 2b ^ 3a · 3b ^ 4 ^ 5a · 5b ^ 6a · 6b · 6c ^ 7a · 7b ^ 8a · 8b · 8c ^ 9 ^ 10 ^ 11 ^ 12 ^ 13 ^ 14 ^ 15
SP1: - - -
SP2: - - - -
SP3: - - -
SP4: - - - - -
SP6: - - - -

Relevant External Links for PRODH Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for PRODH Gene

mRNA expression in normal human tissues for PRODH Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Protein differential expression in normal tissues for PRODH Gene

This gene is overexpressed in Bone (14.4), Brain (11.4), Frontal cortex (9.3), Fetal gut (7.4), Breast (7.3), and Spinal cord (6.2).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for PRODH Gene

SOURCE GeneReport for Unigene cluster for PRODH Gene Hs.517352

mRNA Expression by UniProt/SwissProt for PRODH Gene

Tissue specificity: Expressed in lung, skeletal muscle and brain, to a lesser extent in heart and kidney, and weakly in liver, placenta and pancreas
genes like me logo Genes that share expressions with PRODH: view

Primer Products

In Situ Assay Products

No data available for mRNA differential expression in normal tissues and Expression partners for PRODH Gene

Orthologs for PRODH Gene

This gene was present in the common ancestor of animals and fungi.

Orthologs for PRODH Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia PRODH 35
  • 84.2 (n)
  • 82.63 (a)
  • 82 (a)
(Canis familiaris)
Mammalia PRODH 35
  • 86.73 (n)
  • 87.96 (a)
  • 74 (a)
(Mus musculus)
Mammalia Prodh 35
  • 82.47 (n)
  • 81.97 (a)
Prodh 16
Prodh 36
  • 81 (a)
(Pan troglodytes)
Mammalia PRODH 35
  • 85.37 (n)
  • 80.53 (a)
(Rattus norvegicus)
Mammalia Prodh 35
  • 81.75 (n)
  • 81.3 (a)
(Ornithorhynchus anatinus)
Mammalia PRODH 36
  • 71 (a)
(Gallus gallus)
Aves PRODH 35
  • 77.49 (n)
  • 75.42 (a)
  • 74 (a)
(Anolis carolinensis)
Reptilia PRODH 36
  • 69 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia LOC100494891 35
  • 67.03 (n)
  • 72.23 (a)
(Danio rerio)
Actinopterygii Dr.12460 35
LOC100537991 35
  • 67.99 (n)
  • 68.97 (a)
PRODH (2 of 3) 36
  • 69 (a)
PRODH (3 of 3) 36
  • 75 (a)
prodha 36
  • 54 (a)
fruit fly
(Drosophila melanogaster)
Insecta slgA 37
  • 47 (a)
slgA 35
  • 56.11 (n)
  • 49.23 (a)
slgA 36
  • 40 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP003860 35
  • 54.46 (n)
  • 49.01 (a)
(Caenorhabditis elegans)
Secernentea B0513.5 37
  • 45 (a)
CELE_B0513.5 35
  • 51.63 (n)
  • 46.83 (a)
B0513.5 36
  • 41 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes PUT1 36
  • 20 (a)
PUT1 38
Species with no ortholog for PRODH:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • oppossum (Monodelphis domestica)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for PRODH Gene

Gene Tree for PRODH (if available)
Gene Tree for PRODH (if available)

Paralogs for PRODH Gene

Paralogs for PRODH Gene

(1) SIMAP similar genes for PRODH Gene using alignment to 5 proteins: Pseudogenes for PRODH Gene

genes like me logo Genes that share paralogs with PRODH: view

Variants for PRODH Gene

Sequence variations from dbSNP and Humsavar for PRODH Gene

SNP ID Clin Chr 22 pos Sequence Context AA Info Type MAF
rs361720 -- 18,915,216(+) GAAGC(C/T)TGCAG intron-variant
rs362229 -- 18,915,187(+) CCCCA(A/G)CCACC intron-variant
rs365169 -- 18,928,813(+) GGTCC(A/C)CCCCC intron-variant
rs367766 -- 18,929,518(+) CCTTC(C/T)GAGAA intron-variant
rs370182 -- 18,918,086(+) GAGCA(A/G)GCTCA intron-variant

Structural Variations from Database of Genomic Variants (DGV) for PRODH Gene

Variant ID Type Subtype PubMed ID
dgv1380e1 CNV Complex 17122850
nsv516997 CNV Gain+Loss 19592680
esv2751935 CNV Loss 17911159
dgv173e55 CNV Gain 17911159
esv32687 CNV Gain+Loss 17666407
esv22247 CNV Gain+Loss 19812545
dgv4821n71 CNV Gain 21882294
dgv4822n71 CNV Gain 21882294
dgv4823n71 CNV Gain 21882294
nsv442503 CNV CNV 18776908
nsv914186 CNV Gain 21882294
dgv4824n71 CNV Gain 21882294
essv17726 CNV CNV 17122850
dgv1381e1 CNV Complex 17122850
dgv1382e1 CNV Complex 17122850
nsv914191 CNV Gain 21882294
dgv4825n71 CNV Gain 21882294
dgv4826n71 CNV Loss 21882294
nsv914195 CNV Loss 21882294
dgv4827n71 CNV Loss 21882294
dgv4828n71 CNV Gain 21882294
dgv4829n71 CNV Gain+Loss 21882294
nsv914209 CNV Gain 21882294
nsv459336 CNV Loss 19166990
dgv541n27 CNV Gain 19166990
dgv542n27 CNV Loss 19166990
nsv471179 CNV Gain 18288195
nsv471180 CNV Loss 18288195
dgv543n27 CNV Gain 19166990
nsv435744 CNV Deletion 17901297
nsv914226 CNV Loss 21882294
nsv914227 CNV Loss 21882294
dgv174e55 CNV Gain 17911159
dgv1383e1 CNV Complex 17122850
nsv914231 CNV Gain 21882294
dgv4830n71 CNV Gain 21882294
dgv4831n71 CNV Gain 21882294
dgv4832n71 CNV Loss 21882294
nsv914244 CNV Loss 21882294
dgv4833n71 CNV Loss 21882294

Relevant External Links for PRODH Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for PRODH Gene

Disorders for PRODH Gene

(2) OMIM Diseases for PRODH Gene (606810)


  • 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:0000269 PubMed: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:0000269 PubMed:11891283}. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry.

(8) Novoseek inferred disease relationships for PRODH Gene

Disease -log(P) Hits PubMed IDs
hyperprolinemia, type i 97.2 3
hyperprolinemia 96.1 8
velocardiofacial syndrome 74.8 1
digeorge syndrome 74.7 2
schizophrenia 66.1 32

Relevant External Links for PRODH

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with PRODH: view

No data available for Genatlas for PRODH Gene

Publications for PRODH Gene

  1. Genetic variation at the 22q11 PRODH2/DGCR6 locus presents an unusual pattern and increases susceptibility to schizophrenia. (PMID: 11891283) Liu H. … Karayiorgou M. (Proc. Natl. Acad. Sci. U.S.A. 2002) 3 4 23 48
  2. PRODH mutations and hyperprolinemia in a subset of schizophrenic patients. (PMID: 12217952) Jacquet H. … Frebourg T. (Hum. Mol. Genet. 2002) 3 4 23 48
  3. The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. (PMID: 10192398) Gogos J.A. … Karayiorgou M. (Nat. Genet. 1999) 2 3 4 23
  4. A quantitative association study between schizotypal traits and COMT, PRODH and BDNF genes in a healthy Chinese population. (PMID: 17604122) Ma X. … Li T. (Psychiatry Res 2007) 3 23 48
  5. A human homologue of the Drosophila melanogaster sluggish-A (proline oxidase) gene maps to 22q11.2, and is a candidate gene for type-I hyperprolinaemia. (PMID: 9385373) Campbell H.D. … Young I.G. (Hum. Genet. 1997) 2 3 4

Products for PRODH Gene

Sources for PRODH Gene

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