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Aliases for DHODH Gene

Aliases for DHODH Gene

  • Dihydroorotate Dehydrogenase (Quinone) 2 3
  • Dihydroorotate Oxidase 3 4
  • DHOdehase 3 4
  • POADS 3 6
  • URA1 3 6
  • Dihydroorotate Dehydrogenase (Quinone), Mitochondrial 3
  • Human Complement Of Yeast URA1 3
  • Dihydroorotate Dehydrogenase 2
  • EC 63
  • EC 4

External Ids for DHODH Gene

Previous GeneCards Identifiers for DHODH Gene

  • GC16P062936
  • GC16P072765
  • GC16P071778
  • GC16P071818
  • GC16P070600
  • GC16P057809

Summaries for DHODH Gene

Entrez Gene Summary for DHODH Gene

  • The protein encoded by this gene catalyzes the fourth enzymatic step, the ubiquinone-mediated oxidation of dihydroorotate to orotate, in de novo pyrimidine biosynthesis. This protein is a mitochondrial protein located on the outer surface of the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]

GeneCards Summary for DHODH Gene

DHODH (Dihydroorotate Dehydrogenase (Quinone)) is a Protein Coding gene. Diseases associated with DHODH include miller syndrome and supernumerary nipples. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include drug binding and ubiquinone binding.

UniProtKB/Swiss-Prot for DHODH Gene

  • Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor

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

Genomics for DHODH Gene

Regulatory Elements for DHODH Gene

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for DHODH Gene

72,008,588 bp from pter
72,027,664 bp from pter
19,077 bases
Plus strand

Genomic View for DHODH Gene

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

RefSeq DNA sequence for DHODH Gene

Proteins for DHODH Gene

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

    Protein Symbol:
    Recommended name:
    Dihydroorotate dehydrogenase (quinone), mitochondrial
    Protein Accession:
    Secondary Accessions:
    • A8K8C8
    • Q6P176

    Protein attributes for DHODH Gene

    395 amino acids
    Molecular mass:
    42867 Da
    Name=FMN; Xref=ChEBI:CHEBI:58210; Note=Binds 1 FMN per subunit.;
    Quaternary structure:
    • Monomer.
    • The identification of DHODH defects as the cause of postaxial acrofacial dysostosis (POADS) was obtained via exome sequencing (PubMed:19915526), demonstrating that this method is a powerful tool for identifying genes underlying rare Mendelian disorders. Exome sequencing consists of targeted resequencing of all protein-coding subsequences, which requires around 5% as much sequencing as a whole human genome.

    Three dimensional structures from OCA and Proteopedia for DHODH Gene

neXtProt entry for DHODH Gene

Proteomics data for DHODH Gene at MOPED

Post-translational modifications for DHODH Gene

  • The uncleaved transit peptide is required for mitochondrial targeting and proper membrane integration
  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for DHODH Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

Domains for DHODH Gene

Suggested Antigen Peptide Sequences for DHODH Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily.
  • Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily.
genes like me logo Genes that share domains with DHODH: view

No data available for Gene Families for DHODH Gene

Function for DHODH Gene

Molecular function for DHODH Gene

GENATLAS Biochemistry:
dihydroorotate dehydrogenase,catalyzing the fourth step of the pyrimidine de novo biosynthesis
UniProtKB/Swiss-Prot CatalyticActivity:
(S)-dihydroorotate + a quinone = orotate + a quinol.
UniProtKB/Swiss-Prot Function:
Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor

Enzyme Numbers (IUBMB) for DHODH Gene

Gene Ontology (GO) - Molecular Function for DHODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004152 dihydroorotate dehydrogenase activity TAS --
GO:0008144 drug binding IEA --
GO:0010181 FMN binding IEA --
GO:0048039 ubiquinone binding IEA --
genes like me logo Genes that share ontologies with DHODH: view

Phenotypes for DHODH Gene

GenomeRNAi human phenotypes for DHODH:
genes like me logo Genes that share phenotypes with DHODH: view

Animal Model Products

miRNA for DHODH Gene

miRTarBase miRNAs that target DHODH

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

Localization for DHODH Gene

Subcellular locations from UniProtKB/Swiss-Prot for DHODH Gene

Mitochondrion inner membrane; Single-pass membrane protein.

Subcellular locations from

Jensen Localization Image for DHODH Gene COMPARTMENTS Subcellular localization image for DHODH gene
Compartment Confidence
mitochondrion 5
cytosol 2
endoplasmic reticulum 1
extracellular 1
lysosome 1
peroxisome 1

Gene Ontology (GO) - Cellular Components for DHODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005654 nucleoplasm IDA --
GO:0005739 mitochondrion IDA --
GO:0005743 mitochondrial inner membrane TAS --
GO:0016020 membrane --
GO:0016021 integral component of membrane IEA --
genes like me logo Genes that share ontologies with DHODH: view

Pathways for DHODH Gene

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

Pathways by source for DHODH Gene

UniProtKB/Swiss-Prot Q02127-PYRD_HUMAN

  • Pathway: Pyrimidine metabolism; UMP biosynthesis via de novo pathway; orotate from (S)-dihydroorotate (quinone route): step 1/1

Interacting Proteins for DHODH Gene

Gene Ontology (GO) - Biological Process for DHODH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006206 pyrimidine nucleobase metabolic process TAS --
GO:0006207 de novo pyrimidine nucleobase biosynthetic process IBA --
GO:0006222 UMP biosynthetic process --
GO:0007565 female pregnancy IEA --
GO:0007595 lactation IEA --
genes like me logo Genes that share ontologies with DHODH: view

Drugs for DHODH Gene

(26) Drugbank Compounds for DHODH Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
  • 5-Methyl-N-(4-(trifluoromethyl)phenyl)-4-isoxazolecarboxamide
75706-12-6 3899 Target inhibitor approved, investigational
  • 2-(trans-4-(P-Chlorophenyl)cyclohexyl)-3-hydroxy-1,4-naphthoquinone
95233-18-4 74989 Target inhibitor approved
  • (Z)-2-Cyano-alpha,alpha,alpha-trifluoro-3-hydroxy-P-crotonotoluidide
163451-81-8 Target inhibitor approved
42617949 Target experimental
42617954 Target experimental

(1) ApexBio Compounds for DHODH Gene

Compound Action Cas Number
Teriflunomide Multiple sclerosis (MS) oral drug [108605-62-5]

(9) HMDB Compounds for DHODH Gene

Compound Synonyms Cas Number PubMed IDs
4,5-Dihydroorotic acid
  • (+)-2,6-dioxo-hexahydro-pyrimidine-4-carboxylate
  • 1H-Purin-6-amine flavin dinucleotide
Flavin Mononucleotide
  • Flanin
Hydrogen peroxide
  • Adeka Super EL

(36) Novoseek inferred chemical compound relationships for DHODH Gene

Compound -log(P) Hits PubMed IDs
dihydroorotate 99.3 197
brequinar sodium 96.1 25
dichloroallyllawsone 95.8 4
redoxal 95.1 7
leflunomide 91.3 33

(1) PharmGKB related drug/compound annotations for DHODH Gene

Drug/compound Annotation
leflunomide Clinical Annotation
genes like me logo Genes that share compounds with DHODH: view

Compound Products

Transcripts for DHODH Gene

Unigene Clusters for DHODH Gene

Dihydroorotate dehydrogenase (quinone):
Representative Sequences:

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

No ASD Table

Relevant External Links for DHODH Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for DHODH Gene

mRNA expression in normal human tissues for DHODH Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

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

This gene is overexpressed in Liver (20.2).

Protein differential expression in normal tissues for DHODH Gene

This gene is overexpressed in Nasal epithelium (61.9).

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

SOURCE GeneReport for Unigene cluster for DHODH Gene Hs.654427

genes like me logo Genes that share expressions with DHODH: view

Expression partners for DHODH Gene

* - Elite partner

Primer Products

In Situ Assay Products

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

Orthologs for DHODH Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for DHODH Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia DHODH 35
  • 87.76 (n)
  • 91.65 (a)
  • 78 (a)
(Canis familiaris)
Mammalia DHODH 35
  • 88.02 (n)
  • 89.62 (a)
  • 84 (a)
(Mus musculus)
Mammalia Dhodh 35
  • 84.81 (n)
  • 87.59 (a)
Dhodh 16
Dhodh 36
  • 88 (a)
(Pan troglodytes)
Mammalia DHODH 35
  • 99.24 (n)
  • 99.49 (a)
  • 99 (a)
(Rattus norvegicus)
Mammalia Dhodh 35
  • 85.15 (n)
  • 88.61 (a)
(Monodelphis domestica)
Mammalia DHODH 36
  • 81 (a)
(Ornithorhynchus anatinus)
Mammalia DHODH 36
  • 83 (a)
(Gallus gallus)
Aves DHODH 35
  • 68.65 (n)
  • 73.13 (a)
  • 73 (a)
(Anolis carolinensis)
Reptilia DHODH 36
  • 73 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia dhodh 35
  • 66.33 (n)
  • 68.53 (a)
Str.12297 35
African clawed frog
(Xenopus laevis)
Amphibia Xl.4872 35
(Danio rerio)
Actinopterygii dhodh 35
  • 65.06 (n)
  • 68.96 (a)
wufc29a01 35
dhodh 36
  • 66 (a)
fruit fly
(Drosophila melanogaster)
Insecta Dhod 37
  • 48 (a)
Dhod 35
  • 53.16 (n)
  • 50.13 (a)
Dhod 36
  • 47 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP002037 35
  • 58.24 (n)
  • 55.49 (a)
(Caenorhabditis elegans)
Secernentea W02D3.2 37
  • 51 (a)
dhod-1 35
  • 51.52 (n)
  • 52.22 (a)
dhod-1 36
  • 48 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_ACL035C 35
  • 52.17 (n)
  • 48.94 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0C09240g 35
  • 50.7 (n)
  • 49.3 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes URA1 36
  • 23 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons PYRD 35
  • 56.04 (n)
  • 54.92 (a)
(Oryza sativa)
Liliopsida Os04g0676300 35
  • 55.1 (n)
  • 56.76 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes ura3 35
  • 50.55 (n)
  • 53.73 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 53 (a)
Species with no ortholog for DHODH:
  • Actinobacteria (Mycobacterium tuberculosis)
  • 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)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • 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)
  • wheat (Triticum aestivum)

Evolution for DHODH Gene

Gene Tree for DHODH (if available)
Gene Tree for DHODH (if available)

Paralogs for DHODH Gene

No data available for Paralogs for DHODH Gene

Variants for DHODH Gene

Sequence variations from dbSNP and Humsavar for DHODH Gene

SNP ID Clin Chr 16 pos Sequence Context AA Info Type MAF
rs752434 -- 72,013,271(-) AGGAC(A/G/T)CCTTA intron-variant
rs1465457 -- 72,012,369(-) CCACT(C/G)CTCAC intron-variant
rs2081222 -- 72,021,750(-) ctggg(C/T)tataa intron-variant
rs2287999 -- 72,025,250(-) AAGTC(C/T)GGGAG utr-variant-3-prime
rs2288000 -- 72,024,982(-) TTGTT(C/T)GTTTG utr-variant-3-prime, downstream-variant-500B

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

Variant ID Type Subtype PubMed ID
nsv7285 OTHER Inversion 18451855
nsv833273 CNV Loss 17160897
dgv898e1 CNV Complex 17122850
nsv428329 CNV Gain 18775914
esv2714683 CNV Deletion 23290073
nsv906894 CNV Gain 21882294

Relevant External Links for DHODH Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

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

Disorders for DHODH Gene

MalaCards: The human disease database

MalaCards: The human disease database.

Search for DHODH Gene in MalaCards »

(4) Diseases for DHODH Gene

(1) OMIM Diseases for DHODH Gene (126064)


  • Postaxial acrofacial dysostosis (POADS) [MIM:263750]: POADS is characterized by severe micrognathia, cleft lip and/or palate, hypoplasia or aplasia of the posterior elements of the limbs, coloboma of the eyelids and supernumerary nipples. POADS is a very rare disorder: only 2 multiplex families, each consisting of 2 affected siblings born to unaffected, nonconsanguineous parents, have been described among a total of around 30 reported cases. {ECO:0000269 PubMed:19915526}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(2) University of Copenhagen DISEASES for DHODH Gene

(8) Novoseek inferred disease relationships for DHODH Gene

Disease -log(P) Hits PubMed IDs
rheumatoid arthritis 49 5
malaria 33.6 6
autoimmune diseases 32.7 2
chagas disease 16 1
virus infection 0 2

Relevant External Links for DHODH

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

No data available for Genatlas for DHODH Gene

Publications for DHODH Gene

  1. Regional mapping of the gene encoding dihydroorotate dehydrogenase, an enzyme involved in UMP synthesis, electron transport, and superoxide generation, to human chromosome region 16q22. (PMID: 8211381) Barnes T. … Patterson D. (Somat. Cell Mol. Genet. 1993) 2 3 23
  2. Requirements for the mitochondrial import and localization of dihydroorotate dehydrogenase. (PMID: 10727948) Rawls J. … Loeffler M. (Eur. J. Biochem. 2000) 3 4 23
  3. Exome sequencing identifies the cause of a Mendelian disorder. (PMID: 19915526) Ng S.B. … Bamshad M.J. (Nat. Genet. 2010) 3 4 23
  4. Dihydroorotate dehydrogenase polymorphism influences the toxicity of leflunomide treatment in patients with rheumatoid arthritis. (PMID: 19605743) Grabar P.B. … Dolzan V. (Ann. Rheum. Dis. 2009) 3 23 48
  5. The effect of exon (19C>A) dihydroorotate dehydrogenase gene polymorphism on rheumatoid arthritis treatment with leflunomide. (PMID: 19207032) Pawlik A. … Drozdzik M. (Pharmacogenomics 2009) 3 23 48

Products for DHODH Gene

Sources for DHODH Gene

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