Free for academic non-profit institutions. Other users need a Commercial license

Aliases for PRODH2 Gene

Aliases for PRODH2 Gene

  • Proline Dehydrogenase 2 2 3 5
  • Kidney And Liver Proline Oxidase 1 3 4
  • Proline Dehydrogenase (Oxidase) 2 2 3
  • Probable Proline Oxidase 2 3 4
  • HSPOX1 3 4
  • Probable Proline Dehydrogenase 2 3
  • Hydroxyproline Dehydrogenase 3
  • EC 61
  • 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 catalyzes the first step in the catabolism of trans-4-hydroxy-L-proline, an amino acid derivative obtained through food intake and collagen turnover. One of the downstream products of this catabolism is glyoxylate, which in people with disorders of glyoxalate metabolism can lead to an increase in oxalate levels and the formation of calcium-oxalate kidney stones. Therefore, this gene may serve as a therapeutic target against primary hyperoxalurias (PH). This gene is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme that catalyzes the first step in proline catabolism. [provided by RefSeq, Jan 2017]

GeneCards Summary for PRODH2 Gene

PRODH2 (Proline Dehydrogenase 2) is a Protein Coding gene. Among its related pathways are Arginine and proline metabolism and 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 CIViC summary , 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 Enhancer Score Enhancer Sources Gene-Enhancer Score TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Gene Targets for Enhancer
GH19G035766 1.1 ENCODE dbSUPER 11.7 +45.4 45449 2.5 KLF1 ZNF792 SAP130 ARID4B THRB ZNF48 ZSCAN9 RARA ZFHX2 GLIS2 ARHGAP33 PRODH2 LINC01529 KIRREL2 NPHS1 UPK1A ENSG00000267049 PROSER3
GH19G035689 0.5 FANTOM5 11 +123.2 123179 0.4 POLR2A ZBTB33 U2AF1L4 UPK1A PRODH2 LRFN3 ATP4A ZBTB32 MAG TYMSP2
GH19G035820 1.1 ENCODE 0.4 -6.9 -6875 0.2 FOXA2 ARID4B FEZF1 ZNF2 ZNF302 ZNF416 ZNF143 ZNF263 SP3 MCM3 ZNF529 ZNF461 ZNF790-AS1 LINC01534 ZNF567 ZFP82 ZFP14 ZNF260 ZNF529-AS1 LINC00665
GH19G035803 1 ENCODE 0.4 +9.3 9322 0.7 PKNOX1 ATF1 ARNT TCF12 ZNF766 ELK1 GATA2 MEF2D CREB3 SMARCA4 TMEM147-AS1 HAUS5 ZNF567 LINC01529 PRODH2
GH19G035845 1.3 Ensembl ENCODE 0.3 -32.7 -32692 1.5 PKNOX1 ATF1 SIN3A DMAP1 ZNF2 ZNF121 GLIS2 KLF7 KLF13 ZNF263 RBM42 ZNF792 ZNF850 ZNF146 KIRREL2 PRODH2
- Elite enhancer and/or Elite enhancer-gene association Download GeneHancer data dump

Enhancers 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

Selected DME Specific Peptides for PRODH2 Gene


Post-translational modifications for PRODH2 Gene

No Post-translational modifications

Other Protein References for PRODH2 Gene

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

Gene Ontology (GO) - Molecular Function for PRODH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004657 proline dehydrogenase activity TAS --
GO:0016491 oxidoreductase activity IEA --
GO:0016645 oxidoreductase activity, acting on the CH-NH group of donors IDA 25697095
GO:0071949 FAD binding IBA --
genes like me logo Genes that share ontologies with PRODH2: view
genes like me logo Genes that share phenotypes with PRODH2: view

Animal Model Products

Inhibitory RNA Products

Clone Products

No data available for Human Phenotype Ontology , Animal Models , miRNA , Transcription Factor Targets and HOMER Transcription for PRODH2 Gene

Localization for PRODH2 Gene

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for PRODH2 gene
Compartment Confidence
mitochondrion 3

Gene Ontology (GO) - Cellular Components for PRODH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA,IBA --
GO:0005743 mitochondrial inner membrane TAS,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.

Gene Ontology (GO) - Biological Process for PRODH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006560 proline metabolic process IEA --
GO:0006562 proline catabolic process TAS,IEA --
GO:0010133 proline catabolic process to glutamate IBA,IEA --
GO:0046487 glyoxylate metabolic process TAS --
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:

Inhibitory RNA Products

Clone Products

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 from GTEx, Illumina, BioGPS, and CGAP SAGE 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, MaxQB, and MOPED for PRODH2 Gene

Protein tissue co-expression partners for PRODH2 Gene

- Elite partner

NURSA nuclear receptor signaling pathways regulating expression of PRODH2 Gene:


SOURCE GeneReport for Unigene cluster for PRODH2 Gene:

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

Primer Products

No data available for mRNA Expression by UniProt/SwissProt , Evidence on tissue expression from TISSUES and Phenotype-based relationships between genes and organs from Gene ORGANizer 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
(Pan troglodytes)
Mammalia PRODH2 34 35
  • 99.19 (n)
(Canis familiaris)
Mammalia PRODH2 34 35
  • 86.55 (n)
(Bos Taurus)
Mammalia PRODH2 34 35
  • 85.28 (n)
(Mus musculus)
Mammalia Prodh2 34 16 35
  • 80.83 (n)
(Rattus norvegicus)
Mammalia Prodh2 34
  • 80.83 (n)
(Monodelphis domestica)
Mammalia PRODH2 35
  • 73 (a)
African clawed frog
(Xenopus laevis)
Amphibia MGC68483 34
(Danio rerio)
Actinopterygii zgc:92040 34
  • 55.58 (n)
  • 41 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes PUT1 35
  • 18 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons ERD5 34
  • 41.18 (n)
(Oryza sativa)
Liliopsida Os10g0550900 34
  • 48.29 (n)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.8337 35
  • 36 (a)
Species where no ortholog for PRODH2 was found in the sources mined by GeneCards:
  • 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
rs1000167577 -- 35,811,371(+) AATAC(A/T)GTAAG intron-variant
rs1000469301 -- 35,801,051(+) AATCC(C/T)GGCTA intron-variant
rs1000490464 -- 35,802,036(+) AGACA(C/T)AGAGA intron-variant
rs1000726139 -- 35,806,188(+) TCAAG(A/T)GATCC intron-variant
rs1001096185 -- 35,806,524(+) TCGTA(C/T)CAGCT reference, missense

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

Variant ID Type Subtype PubMed ID
esv2718500 CNV deletion 23290073
esv3583358 CNV loss 25503493
esv3583362 CNV loss 25503493
nsv953286 CNV deletion 24416366

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)

Relevant External Links for PRODH2 Gene

Human Gene Mutation Database (HGMD)
SNPedia medical, phenotypic, and genealogical associations of SNPs for

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for PRODH2 Gene

Disorders for PRODH2 Gene

Relevant External Links for PRODH2

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:

No disorders were found for PRODH2 Gene.

No data available for MalaCards , 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 46 64
  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 64
  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 64
  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 64
  5. Genetic study of schizophrenia: new targets for therapy. (PMID: 19810846) Ujike H. … Nakata K. (Expert Rev Neurother 2003) 22 64

Products for PRODH2 Gene

Sources for PRODH2 Gene

Loading form....