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Aliases for PRODH2 Gene

Aliases for PRODH2 Gene

  • Proline Dehydrogenase 2 2 3
  • Proline Dehydrogenase (Oxidase) 2 2 3 5
  • Kidney And Liver Proline Oxidase 1 3 4
  • Probable Proline Oxidase 2 3 4
  • HSPOX1 3 4
  • EC 63
  • EC 4
  • HYPDH 3

External Ids for PRODH2 Gene

Previous GeneCards Identifiers for PRODH2 Gene

  • GC19M036930
  • GC19M036739
  • GC19M040967
  • GC19M040982
  • GC19M036291
  • GC19M036292
  • GC19M032795
  • GC19M036416
  • GC19M036543
  • GC19M037061

Summaries for PRODH2 Gene

Entrez Gene Summary for PRODH2 Gene

  • The protein encoded by this gene is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme which catalyzes the first step in proline catabolism. The function of this protein has not been determined. [provided by RefSeq, Jul 2008]

GeneCards Summary for PRODH2 Gene

PRODH2 (Proline Dehydrogenase 2) is a Protein Coding gene. Diseases associated with PRODH2 include Schizophrenia 4 and Hyperprolinemia, Type I. Among its related pathways are Metabolism and Arginine and proline metabolism. GO annotations related to this gene include proline dehydrogenase activity. An important paralog of this gene is PRODH.

UniProtKB/Swiss-Prot for PRODH2 Gene

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

No data available for Tocris Summary , Gene Wiki entry , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for PRODH2 Gene

Genomics for PRODH2 Gene

Regulatory Elements for PRODH2 Gene

Enhancers for PRODH2 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around PRODH2 on UCSC Golden Path with GeneCards custom track

Promoters for PRODH2 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around PRODH2 on UCSC Golden Path with GeneCards custom track

Genomic Location for PRODH2 Gene

35,799,988 bp from pter
35,813,299 bp from pter
13,312 bases
Minus strand

Genomic View for PRODH2 Gene

Genes around PRODH2 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
PRODH2 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for PRODH2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for PRODH2 Gene

Proteins for PRODH2 Gene

  • Protein details for PRODH2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Probable proline dehydrogenase 2
    Protein Accession:

    Protein attributes for PRODH2 Gene

    536 amino acids
    Molecular mass:
    58871 Da
    Name=FAD; Xref=ChEBI:CHEBI:57692;
    Quaternary structure:
    No Data Available

neXtProt entry for PRODH2 Gene

Proteomics data for PRODH2 Gene at MOPED

Selected DME Specific Peptides for PRODH2 Gene

Post-translational modifications for PRODH2 Gene

No Post-translational modifications

Other Protein References for PRODH2 Gene

ENSEMBL proteins:
REFSEQ proteins:

Antibody Products

Domains & Families for PRODH2 Gene

Protein Domains for PRODH2 Gene

Suggested Antigen Peptide Sequences for PRODH2 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 PRODH2: view

No data available for Gene Families for PRODH2 Gene

Function for PRODH2 Gene

Molecular function for PRODH2 Gene

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.

Enzyme Numbers (IUBMB) for PRODH2 Gene

genes like me logo Genes that share phenotypes with PRODH2: view

Animal Model Products

No data available for Gene Ontology (GO) - Molecular Function , Human Phenotype Ontology , Animal Models , miRNA , Transcription Factor Targets and HOMER Transcription for PRODH2 Gene

Localization for PRODH2 Gene

Subcellular locations from

Jensen Localization Image for PRODH2 Gene COMPARTMENTS Subcellular localization image for PRODH2 gene
Compartment Confidence
mitochondrion 2

Gene Ontology (GO) - Cellular Components for PRODH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005743 mitochondrial inner membrane IEA --
genes like me logo Genes that share ontologies with PRODH2: view

No data available for Subcellular locations from UniProtKB/Swiss-Prot for PRODH2 Gene

Pathways & Interactions for PRODH2 Gene

genes like me logo Genes that share pathways with PRODH2: view

Pathways by source for PRODH2 Gene

1 BioSystems pathway for PRODH2 Gene
2 KEGG pathways for PRODH2 Gene

UniProtKB/Swiss-Prot Q9UF12-PROD2_HUMAN

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

Interacting Proteins for PRODH2 Gene

Gene Ontology (GO) - Biological Process for PRODH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006562 proline catabolic process IEA --
GO:0055114 oxidation-reduction process IEA --
genes like me logo Genes that share ontologies with PRODH2: view

No data available for SIGNOR curated interactions for PRODH2 Gene

Drugs & Compounds for PRODH2 Gene

(2) Drugs for PRODH2 Gene - From: HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
FAD Approved Pharma 0
L-Proline Approved Nutra 0

(1) Additional Compounds for PRODH2 Gene - From: HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
1-Pyrroline-5-carboxylic acid
  • (S)-1-pyrroline-5-carboxylate
  • 1-Pyrroline-5-carboxylate
  • 1-Pyrroline-5-carboxylic acid
  • 3,4-Dihydro-2h-pyrrole-2-carboxylate
  • 3,4-Dihydro-2h-pyrrole-2-carboxylic acid
genes like me logo Genes that share compounds with PRODH2: view

Transcripts for PRODH2 Gene

Unigene Clusters for PRODH2 Gene

Proline dehydrogenase (oxidase) 2:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for PRODH2 Gene

No ASD Table

Relevant External Links for PRODH2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for PRODH2 Gene

mRNA expression in normal human tissues for PRODH2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for PRODH2 Gene

This gene is overexpressed in Liver (x28.9) and Kidney - Cortex (x22.7).

Protein differential expression in normal tissues from HIPED for PRODH2 Gene

This gene is overexpressed in Kidney (48.0) and Gallbladder (15.7).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for PRODH2 Gene

SOURCE GeneReport for Unigene cluster for PRODH2 Gene Hs.515366

genes like me logo Genes that share expression patterns with PRODH2: view

Protein tissue co-expression partners for PRODH2 Gene

- Elite partner

Primer Products

No data available for mRNA Expression by UniProt/SwissProt for PRODH2 Gene

Orthologs for PRODH2 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for PRODH2 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia PRODH2 35
  • 85.28 (n)
  • 85.2 (a)
  • 84 (a)
(Canis familiaris)
Mammalia PRODH2 35
  • 86.55 (n)
  • 85.2 (a)
  • 84 (a)
(Mus musculus)
Mammalia Prodh2 35
  • 80.83 (n)
  • 80.97 (a)
Prodh2 16
Prodh2 36
  • 80 (a)
(Pan troglodytes)
Mammalia PRODH2 35
  • 99.19 (n)
  • 99.07 (a)
  • 98 (a)
(Rattus norvegicus)
Mammalia Prodh2 35
  • 80.83 (n)
  • 80.75 (a)
(Monodelphis domestica)
Mammalia PRODH2 36
  • 73 (a)
African clawed frog
(Xenopus laevis)
Amphibia MGC68483 35
(Danio rerio)
Actinopterygii zgc:92040 35
  • 55.58 (n)
  • 50 (a)
  • 41 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes PUT1 36
  • 18 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons ERD5 35
  • 41.18 (n)
  • 31.13 (a)
(Oryza sativa)
Liliopsida Os10g0550900 35
  • 48.29 (n)
  • 37.32 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.8337 36
  • 36 (a)
Species with no ortholog for PRODH2:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • 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)
  • chicken (Gallus gallus)
  • 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)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • lizard (Anolis carolinensis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • platypus (Ornithorhynchus anatinus)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • tropical clawed frog (Silurana tropicalis)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)

Evolution for PRODH2 Gene

Gene Tree for PRODH2 (if available)
Gene Tree for PRODH2 (if available)

Paralogs for PRODH2 Gene

Paralogs for PRODH2 Gene

(1) SIMAP similar genes for PRODH2 Gene using alignment to 4 proteins:

genes like me logo Genes that share paralogs with PRODH2: view

Variants for PRODH2 Gene

Sequence variations from dbSNP and Humsavar for PRODH2 Gene

SNP ID Clin Chr 19 pos Sequence Context AA Info Type
rs3848666 - 35,812,762(+) TGGAG(C/G/T)GGGGA reference, missense
rs3761097 - 35,800,075(-) GTGGC(A/C/G)GCGGC reference, missense
rs7257630 -- 35,813,967(+) tagta(A/G)agaca upstream-variant-2KB
rs7260272 -- 35,802,754(+) ATGGG(G/T)TCTTG intron-variant
rs7507463 -- 35,809,166(+) ctttc(C/T)ttctt intron-variant

Structural Variations from Database of Genomic Variants (DGV) for PRODH2 Gene

Variant ID Type Subtype PubMed ID
dgv3852n71 CNV Loss 21882294
dgv3853n71 CNV Loss 21882294
nsv911636 CNV Loss 21882294
nsv911637 CNV Loss 21882294
nsv911638 CNV Loss 21882294
esv2718500 CNV Deletion 23290073

Variation tolerance for PRODH2 Gene

Residual Variation Intolerance Score: 92.4% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 4.92; 67.77% of all genes are more intolerant (likely to be disease-causing)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot and Relevant External Links for PRODH2 Gene

Disorders for PRODH2 Gene

MalaCards: The human disease database

(2) MalaCards diseases for PRODH2 Gene - From: OMIM

Disorder Aliases PubMed IDs
schizophrenia 4
  • schizophrenia, susceptibility to, 4
hyperprolinemia, type i
  • proline oxidase deficiency
- elite association - COSMIC cancer census association via MalaCards

Relevant External Links for PRODH2

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with PRODH2: view

No data available for UniProtKB/Swiss-Prot and Genatlas for PRODH2 Gene

Publications for PRODH2 Gene

  1. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson S.L. … O'Brien S.J. (PLoS ONE 2010) 3 48 67
  2. Proline dehydrogenase 2 (PRODH2) is a hydroxyproline dehydrogenase (HYPDH) and molecular target for treating primary hyperoxaluria. (PMID: 25697095) Summitt C.B. … Lowther W.T. (Biochem. J. 2015) 3
  3. P53 family members modulate the expression of PRODH, but not PRODH2, via intronic p53 response elements. (PMID: 23861960) Raimondi I. … Campomenosi P. (PLoS ONE 2013) 3
  4. A novel function for hydroxyproline oxidase in apoptosis through generation of reactive oxygen species. (PMID: 18287100) Cooper S.K. … Phang J.M. (J. Biol. Chem. 2008) 3
  5. Genetic study of schizophrenia: new targets for therapy. (PMID: 19810846) Ujike H. … Nakata K. (Expert Rev Neurother 2003) 23

Products for PRODH2 Gene

Sources for PRODH2 Gene