UniProt Accession

 D2HDH_HUMAN; Q8N465; Q6IQ24; Q8N5Q8 

Genbank Protein ID

 AC114730; BC036604; BC071598 

Genbank Nucleotide ID

 AAX82020.1; AAH36604.2; AAH71598.1 

Protein Name

 D-2-hydroxyglutarate dehydrogenase, mitochondrial 

Protein Synonyms/Alias

  

Gene Name

 D2HGDH 

Gene Synonyms/Alias

 D2HGD 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
64	LPFSTVSKQDLAAFE	ubiquitination	[1, 2, 3]

Reference

 [1] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [2] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [3] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 

Functional Description

 Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate. 

Sequence Annotation

 DOMAIN 96 275 FAD-binding PCMH-type.  

Keyword

 Alternative splicing; Complete proteome; Disease mutation; FAD; Flavoprotein; Mitochondrion; Oxidoreductase; Polymorphism; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 521 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0051990; F:(R)-2-hydroxyglutarate dehydrogenase activity; ISS:HGNC.
 GO:0050660; F:flavin adenine dinucleotide binding; IEA:InterPro.
 GO:0008762; F:UDP-N-acetylmuramate dehydrogenase activity; IEA:InterPro.
 GO:0006103; P:2-oxoglutarate metabolic process; TAS:Reactome.
 GO:0044267; P:cellular protein metabolic process; ISS:HGNC.
 GO:0032025; P:response to cobalt ion; ISS:HGNC.
 GO:0010042; P:response to manganese ion; ISS:HGNC.
 GO:0010043; P:response to zinc ion; ISS:HGNC. 

Interpro

 IPR016169; CO_DH_flavot_FAD-bd_sub2.
 IPR016166; FAD-bd_2.
 IPR016167; FAD-bd_2_sub1.
 IPR016164; FAD-linked_Oxase-like_C.
 IPR004113; FAD-linked_oxidase_C.
 IPR006094; Oxid_FAD_bind_N.
 IPR016171; Vanillyl_alc_oxidase_C-sub2. 

Pfam

 PF02913; FAD-oxidase_C
 PF01565; FAD_binding_4 

SMART

  

PROSITE

 PS51387; FAD_PCMH 

PRINTS