• Gene id: 1723

Gene Summary

• Gene Symbol: DHODH   Gene   UCSC   Ensembl
• Aliases: DHOdehase, POADS, URA1
• Gene name: dihydroorotate dehydrogenase (quinone)
• Alternate names: dihydroorotate dehydrogenase (quinone), mitochondrial, dihydroorotate oxidase, human complement of yeast URA1,
• Gene location: 16q22.2 (72008743: 72027658)     Exons: 11     NC_000016.10
• Gene summary(Entrez): 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 mit
• OMIM: 613332

Protein Summary

• Protein general information: Q02127  
  • Name: Dihydroorotate dehydrogenase (quinone), mitochondrial (DHOdehase) (EC 1.3.5.2) (Dihydroorotate oxidase)
  • Length: 395  
  • Mass: 42867
• Sequence:

  MAWRHLKKRAQDAVIILGGGGLLFASYLMATGDERFYAEHLMPTLQGLLDPESAHRLAVRFTSLGLLPRARFQDS
  DMLEVRVLGHKFRNPVGIAAGFDKHGEAVDGLYKMGFGFVEIGSVTPKPQEGNPRPRVFRLPEDQAVINRYGFNS
  HGLSVVEHRLRARQQKQAKLTEDGLPLGVNLGKNKTSVDAAEDYAEGVRVLGPLADYLVVNVSSPNTAGLRSLQG
  KAELRRLLTKVLQERDGLRRVHRPAVLVKIAPDLTSQDKEDIASVVKELGIDGLIVTNTTVSRPAGLQGALRSET
  GGLSGKPLRDLSTQTIREMYALTQGRVPIIGVGGVSSGQDALEKIRAGASLVQLYTALTFWGPPVVGKVKRELEA
  LLKEQGFGGVTDAIGADHRR

• Structural information:
  • Interpro: IPR013785  IPR005720  IPR005719  IPR001295  
  • Prosite: PS00911 PS00912
  • CDD: cd04738
  • PDB: 1D3G 1D3H 2B0M 2BXV 2FPT 2FPV 2FPY 2FQI 2PRH 2PRL 2PRM 2WV8 3F1Q 3FJ6 3FJL 3G0U 3G0X 3KVJ 3KVK 3KVL 3KVM 3U2O 3W7R 3ZWS 3ZWT 4IGH 4JGD 4JS3 4JTS 4JTT 4JTU 4LS0 4LS1 4LS2 4OQV 4RK8 4RKA 4RLI 4RR4 4YLW 4ZL1 4ZMG 5H2Z 5H73 5HIN 5HQE 5K9C 5K9D 5MUT 5MVC 5MVD 5TCE 5ZF4 5ZF7 5ZF8 5ZF9 5ZFA 5ZFB 6CJF 6CJG 6ET4 6FMD 6GK0 6ID
  • PDBsum: 1D3G 1D3H 2B0M 2BXV 2FPT 2FPV 2FPY 2FQI 2PRH 2PRL 2PRM 2WV8 3F1Q 3FJ6 3FJL 3G0U 3G0X 3KVJ 3KVK 3KVL 3KVM 3U2O 3W7R 3ZWS 3ZWT 4IGH 4JGD 4JS3 4JTS 4JTT 4JTU 4LS0 4LS1 4LS2 4OQV 4RK8 4RKA 4RLI 4RR4 4YLW 4ZL1 4ZMG 5H2Z 5H73 5HIN 5HQE 5K9C 5K9D 5MUT 5MVC 5MVD 5TCE 5ZF4 5ZF7 5ZF8 5ZF9 5ZFA 5ZFB 6CJF 6CJG 6ET4 6FMD 6GK0 6ID
  • STRING: ENSP00000219240
• Other Databases: GeneCards:  DHODH  Malacards:  DHODH

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0004152	dihydroorotate dehydrogenase activity	IBA	molecular function
GO:0005739	mitochondrion	IBA	cellular component
GO:0005743	mitochondrial inner membrane	IBA	cellular component
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IBA	biological process
GO:0009220	pyrimidine ribonucleotide biosynthetic process	IBA	biological process
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0003824	catalytic activity	IEA	molecular function
GO:0005737	cytoplasm	IEA	cellular component
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IEA	biological process
GO:0016020	membrane	IEA	cellular component
GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0006221	pyrimidine nucleotide biosynthetic process	IEA	biological process
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0016020	membrane	IEA	cellular component
GO:0016491	oxidoreductase activity	IEA	molecular function
GO:0005739	mitochondrion	IEA	cellular component
GO:0016021	integral component of membrane	IEA	cellular component
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:0005743	mitochondrial inner membrane	TAS	cellular component
GO:0046134	pyrimidine nucleoside biosynthetic process	TAS	biological process
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0042594	response to starvation	IEA	biological process
GO:0014070	response to organic cyclic compound	IEA	biological process
GO:0010181	FMN binding	IEA	molecular function
GO:0007595	lactation	IEA	biological process
GO:0007565	female pregnancy	IEA	biological process
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:1903576	response to L-arginine	IEA	biological process
GO:0090140	regulation of mitochondrial fission	IEA	biological process
GO:0048039	ubiquinone binding	IEA	molecular function
GO:0043065	positive regulation of apoptotic process	IEA	biological process
GO:0043025	neuronal cell body	IEA	cellular component
GO:0042493	response to drug	IEA	biological process
GO:0031000	response to caffeine	IEA	biological process
GO:0008144	drug binding	IEA	molecular function
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IEA	biological process
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0005739	mitochondrion	IDA	cellular component
GO:0005654	nucleoplasm	IDA	cellular component
GO:0005829	cytosol	IDA	cellular component
GO:0044205	'de novo' UMP biosynthetic process	IEA	biological process
GO:0004152	dihydroorotate dehydrogenase activity	IBA	molecular function
GO:0005739	mitochondrion	IBA	cellular component
GO:0005743	mitochondrial inner membrane	IBA	cellular component
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IBA	biological process
GO:0009220	pyrimidine ribonucleotide biosynthetic process	IBA	biological process
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0003824	catalytic activity	IEA	molecular function
GO:0005737	cytoplasm	IEA	cellular component
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IEA	biological process
GO:0016020	membrane	IEA	cellular component
GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0006221	pyrimidine nucleotide biosynthetic process	IEA	biological process
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0016020	membrane	IEA	cellular component
GO:0016491	oxidoreductase activity	IEA	molecular function
GO:0005739	mitochondrion	IEA	cellular component
GO:0016021	integral component of membrane	IEA	cellular component
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:0005743	mitochondrial inner membrane	TAS	cellular component
GO:0046134	pyrimidine nucleoside biosynthetic process	TAS	biological process
GO:0005515	protein binding	IPI	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0042594	response to starvation	IEA	biological process
GO:0014070	response to organic cyclic compound	IEA	biological process
GO:0010181	FMN binding	IEA	molecular function
GO:0007595	lactation	IEA	biological process
GO:0007565	female pregnancy	IEA	biological process
GO:0004152	dihydroorotate dehydrogenase activity	IEA	molecular function
GO:1903576	response to L-arginine	IEA	biological process
GO:0090140	regulation of mitochondrial fission	IEA	biological process
GO:0048039	ubiquinone binding	IEA	molecular function
GO:0043065	positive regulation of apoptotic process	IEA	biological process
GO:0043025	neuronal cell body	IEA	cellular component
GO:0042493	response to drug	IEA	biological process
GO:0031000	response to caffeine	IEA	biological process
GO:0008144	drug binding	IEA	molecular function
GO:0006207	'de novo' pyrimidine nucleobase biosynthetic process	IEA	biological process
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0005743	mitochondrial inner membrane	IEA	cellular component
GO:0005739	mitochondrion	IDA	cellular component
GO:0005654	nucleoplasm	IDA	cellular component
GO:0005829	cytosol	IDA	cellular component
GO:0044205	'de novo' UMP biosynthetic process	IEA	biological process

KEGG pathways

Pathway id	Pathway name
hsa01100	Metabolic pathways
hsa00240	Pyrimidine metabolism

Diseases

Associated diseases	References
Acrofacial dysostosis	KEGG:H01376
Acrofacial dysostosis	KEGG:H01376
Aberrant CpGs in Low Motility Sperm	MIK: 21674046

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
21674046	Aberrant CpGs in Low Motility Sperm			18	Male infertility	GSE26881