D2hgdh Gene Summary [Mouse]

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 2025]

Details

Type
Protein Coding
Official Symbol
D2hgdh
Official Name
D-2-hydroxyglutarate dehydrogenase [Source:MGI Symbol;Acc:MGI:2138209]
Ensembl ID
ENSMUSG00000073609
Bio databases IDs NCBI: 98314 Ensembl: ENSMUSG00000073609
Aliases D-2-hydroxyglutarate dehydrogenase
Synonyms AA408776, AA408778, D2HGD, D-2-hydroxyglutarate dehydrogenase, LOC257054, RGD1307976
Species
Mouse, Mus musculus
OrthologiesHumanRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse D2hgdh often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • FAD binding domain
  • zinc ion binding
  • FAD linked oxidases, C-terminal domain
  • CH-OH group oxidoreductase
  • enzyme

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • colitis
  • D-2-hydroxyglutaric aciduria type 1
  • allergy
  • asthma
  • respiratory disorder
  • hereditary disorder
  • D-2-hydroxyglutaric aciduria
  • eczema
  • allergic rhinitis
  • glioma
regulated by
regulates
  • 2-oxoglutaric acid
  • malic acid

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • Mitochondria
  • mitochondrial matrix

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the mouse D2hgdh gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • response to manganese ion
  • protein metabolic process
  • malate metabolic process
  • response to zinc ion
  • response to calcium ion
  • response to cobalt ion
  • response to magnesium ion
  • 2-oxoglutarate metabolic process

Cellular Component

Where in the cell the gene product is active
  • mitochondrial matrix
  • mitochondrion

Molecular Function

What the gene product does at the molecular level
  • FAD binding
  • zinc ion binding
  • (R)-2-hydroxyglutarate dehydrogenase activity

Gene-Specific Assays for Results You Can Trust

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