U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

D2hgdh D-2-hydroxyglutarate dehydrogenase [ Mus musculus (house mouse) ]

Gene ID: 98314, updated on 1-Mar-2024

Summary

Official Symbol
D2hgdhprovided by MGI
Official Full Name
D-2-hydroxyglutarate dehydrogenaseprovided by MGI
Primary source
MGI:MGI:2138209
See related
Ensembl:ENSMUSG00000073609 AllianceGenome:MGI:2138209
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Summary
Predicted to enable (R)-2-hydroxyglutarate dehydrogenase activity and zinc ion binding activity. Predicted to be involved in several processes, including malate metabolic process; response to cobalt ion; and response to manganese ion. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in D-2-hydroxyglutaric aciduria 1. Orthologous to human D2HGDH (D-2-hydroxyglutarate dehydrogenase). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Ubiquitous expression in ovary adult (RPKM 9.9), kidney adult (RPKM 9.3) and 28 other tissues See more
Orthologs
NEW
Try the new Gene table
Try the new Transcript table

Genomic context

See D2hgdh in Genome Data Viewer
Location:
1 D; 1 47.36 cM
Exon count:
12
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 1 NC_000067.7 (93752531..93781583)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 1 NC_000067.6 (93823195..93853857)

Chromosome 1 - NC_000067.7Genomic Context describing neighboring genes Neighboring gene CapStarr-seq enhancer MGSCv37_chr1:95698432-95698719 Neighboring gene STARR-positive B cell enhancer ABC_E4338 Neighboring gene deoxythymidylate kinase Neighboring gene predicted gene, 22089 Neighboring gene inhibitor of growth family, member 5 Neighboring gene STARR-positive B cell enhancer ABC_E7734 Neighboring gene galactose-3-O-sulfotransferase 2 Neighboring gene STARR-seq mESC enhancer starr_01736 Neighboring gene STARR-seq mESC enhancer starr_01737 Neighboring gene STARR-seq mESC enhancer starr_01738 Neighboring gene D-2-hydroxyglutarate dehydrogenase pseudogene

Genomic regions, transcripts, and products

Expression

  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Variation

Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Gene trapped (1) 
  • Endonuclease-mediated (1) 

Pathways from PubChem

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables (R)-2-hydroxyglutarate dehydrogenase activity ISO
Inferred from Sequence Orthology
more info
 
enables (R)-2-hydroxyglutarate dehydrogenase activity ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
enables FAD binding IEA
Inferred from Electronic Annotation
more info
 
enables catalytic activity IEA
Inferred from Electronic Annotation
more info
 
enables flavin adenine dinucleotide binding IEA
Inferred from Electronic Annotation
more info
 
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
 
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
 
enables zinc ion binding ISO
Inferred from Sequence Orthology
more info
 
Process Evidence Code Pubs
involved_in malate metabolic process ISO
Inferred from Sequence Orthology
more info
 
involved_in protein metabolic process ISO
Inferred from Sequence Orthology
more info
 
involved_in protein metabolic process ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
NOT involved_in response to calcium ion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
involved_in response to cobalt ion ISO
Inferred from Sequence Orthology
more info
 
involved_in response to cobalt ion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
NOT involved_in response to magnesium ion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
involved_in response to manganese ion ISO
Inferred from Sequence Orthology
more info
 
involved_in response to manganese ion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
involved_in response to zinc ion ISO
Inferred from Sequence Orthology
more info
 
involved_in response to zinc ion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
Component Evidence Code Pubs
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
 
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
 
located_in mitochondrion ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 

General protein information

Preferred Names
D-2-hydroxyglutarate dehydrogenase, mitochondrial
NP_001297696.1
NP_001408163.1
NP_001408164.1
NP_001408165.1
NP_001408166.1
NP_001408167.1
NP_001408168.1
NP_001408169.1
NP_001408170.1
NP_849213.2
XP_006530033.2
XP_006530036.1
XP_017168525.1
XP_030099718.1
XP_036010489.1

NCBI Reference Sequences (RefSeq)

NEW Try the new Transcript table

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001310767.2NP_001297696.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 1 precursor

    Status: VALIDATED

    Source sequence(s)
    AC167139
    Consensus CDS
    CCDS78658.1
    UniProtKB/TrEMBL
    E9QN44
    Related
    ENSMUSP00000108502.2, ENSMUST00000112881.8
    Conserved Domains (3) summary
    COG0277
    Location:112554
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]
    pfam01565
    Location:137274
    FAD_binding_4; FAD binding domain
    pfam02913
    Location:311551
    FAD-oxidase_C; FAD linked oxidases, C-terminal domain
  2. NM_001421234.1NP_001408163.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 2 precursor

    Status: VALIDATED

    Source sequence(s)
    AC167139
    UniProtKB/Swiss-Prot
    E9QLL1, Q149H0, Q3TDF5, Q8BU06, Q8CIM3
  3. NM_001421235.1NP_001408164.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 3 precursor

    Status: VALIDATED

    Source sequence(s)
    AC167139
  4. NM_001421236.1NP_001408165.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 4 precursor

    Status: VALIDATED

    Source sequence(s)
    AC167139
  5. NM_001421237.1NP_001408166.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 5

    Status: VALIDATED

    Source sequence(s)
    AC167139
  6. NM_001421238.1NP_001408167.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 5

    Status: VALIDATED

    Source sequence(s)
    AC167139
  7. NM_001421239.1NP_001408168.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 6

    Status: VALIDATED

    Source sequence(s)
    AC167139
  8. NM_001421240.1NP_001408169.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 6

    Status: VALIDATED

    Source sequence(s)
    AC167139
  9. NM_001421241.1NP_001408170.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 6

    Status: VALIDATED

    Source sequence(s)
    AC167139
  10. NM_178882.5NP_849213.2  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 2 precursor

    See identical proteins and their annotated locations for NP_849213.2

    Status: VALIDATED

    Source sequence(s)
    AC167139
    Consensus CDS
    CCDS15196.1
    UniProtKB/Swiss-Prot
    E9QLL1, Q149H0, Q3TDF5, Q8BU06, Q8CIM3
    Related
    ENSMUSP00000095235.4, ENSMUST00000097633.10
    Conserved Domains (1) summary
    COG0277
    Location:90532
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000067.7 Reference GRCm39 C57BL/6J

    Range
    93752531..93781583
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006529970.4XP_006530033.2  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X2

    Conserved Domains (1) summary
    COG0277
    Location:90396
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]
  2. XM_030243858.2XP_030099718.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X4

    Conserved Domains (1) summary
    COG0277
    Location:112418
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]
  3. XM_017313036.3XP_017168525.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X3

    Conserved Domains (1) summary
    COG0277
    Location:1429
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]
  4. XM_006529973.5XP_006530036.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X6

    See identical proteins and their annotated locations for XP_006530036.1

    Conserved Domains (2) summary
    PRK11282
    Location:2477
    glcE; glycolate oxidase FAD binding subunit; Provisional
    pfam02913
    Location:52291
    FAD-oxidase_C; FAD linked oxidases, C-terminal domain
  5. XM_036154596.1XP_036010489.1  D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X3

    Conserved Domains (1) summary
    COG0277
    Location:1429
    GlcD; FAD/FMN-containing dehydrogenase [Energy production and conversion]