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L2HGDH L-2-hydroxyglutarate dehydrogenase [ Drosophila melanogaster (fruit fly) ]

Gene ID: 35156, updated on 10-May-2024

Summary

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Official Symbol
L2HGDHprovided by FlyBase
Official Full Name
L-2-hydroxyglutarate dehydrogenaseprovided by FlyBase
Primary source
FLYBASE:FBgn0032729
Locus tag
Dmel_CG10639
See related
AllianceGenome:FB:FBgn0032729
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Drosophila melanogaster
Lineage
Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
Also known as
CG10639; dL2HGDH; Dmel\CG10639; L-2-HGDH
Summary
Predicted to enable (S)-2-hydroxy-acid oxidase activity and 2-hydroxyglutarate dehydrogenase activity. Predicted to be active in mitochondrion. Is expressed in adult head and adult heart. Human ortholog(s) of this gene implicated in 2-hydroxyglutaric aciduria; L-2-hydroxyglutaric aciduria; cerebellar ataxia; hereditary spastic paraplegia; and visual epilepsy. Orthologous to human L2HGDH (L-2-hydroxyglutarate dehydrogenase). [provided by Alliance of Genome Resources, Apr 2022]
Orthologs
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Genomic context

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Location:
37B8-37B8; 2-54 cM
Exon count:
3
Annotation release Status Assembly Chr Location
Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 2L NT_033779.5 (18971993..18974448, complement)
Release 5.57 previous assembly Release 5 (GCF_000001215.2) 2L NT_033779.4 (18971993..18974448, complement)

Chromosome 2L - NT_033779.5Genomic Context describing neighboring genes Neighboring gene Betaine-homocysteine S-methyltransferase Neighboring gene Numb-associated kinase Neighboring gene Transport and Golgi organization 6 Neighboring gene uncharacterized protein Neighboring gene Swiprosin-1 Neighboring gene uncharacterized protein Neighboring gene ER membrane protein complex subunit 5

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. The oncometabolite L-2-hydroxyglutarate is a common product of dipteran larval development. Mahmoudzadeh NH, et al. Insect Biochem Mol Biol, 2020 Dec. PMID 33157229, Free PMC Article
  2. Drosophila larvae synthesize the putative oncometabolite L-2-hydroxyglutarate during normal developmental growth. Li H, et al. Proc Natl Acad Sci U S A, 2017 Feb 7. PMID 28115720, Free PMC Article
  3. A Drosophila model of combined D-2- and L-2-hydroxyglutaric aciduria reveals a mechanism linking mitochondrial citrate export with oncometabolite accumulation. Li H, et al. Dis Model Mech, 2018 Sep 21. PMID 30108060, Free PMC Article
  4. Mitochondrial stress causes neuronal dysfunction via an ATF4-dependent increase in L-2-hydroxyglutarate. Hunt RJ, et al. J Cell Biol, 2019 Dec 2. PMID 31645461, Free PMC Article
  5. Glial Metabolic Rewiring Promotes Axon Regeneration and Functional Recovery in the Central Nervous System. Li F, et al. Cell Metab, 2020 Nov 3. PMID 32941799, Free PMC Article

See all (32) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Structure and biochemical characterization of l-2-hydroxyglutarate dehydrogenase and its role in the pathogenesis of l-2-hydroxyglutaric aciduria.
    Title: Structure and biochemical characterization of l-2-hydroxyglutarate dehydrogenase and its role in the pathogenesis of l-2-hydroxyglutaric aciduria.
Submit: New GeneRIF Correction

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Homology

Gene Ontology Provided by FlyBase

Function Evidence Code Pubs
enables 2-hydroxyglutarate dehydrogenase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables 2-hydroxyglutarate dehydrogenase activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables 2-hydroxyglutarate dehydrogenase activity, forward reaction IMP
Inferred from Mutant Phenotype
more info
 PubMed 
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
  

General protein information

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Preferred Names
L-2-hydroxyglutarate dehydrogenase
Names
CG10639-PA
CG10639-PB
CG10639-PC
L2HGDH-PA
L2HGDH-PB
L2HGDH-PC
NP_001286076.1
NP_001286077.1
NP_609923.2

NCBI Reference Sequences (RefSeq)

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Genome Annotation

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

Reference assembly

Genomic

  1. NT_033779.5 Reference assembly

    Range
    18971993..18974448 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_136079.3 → NP_609923.2  L-2-hydroxyglutarate dehydrogenase, isoform A [Drosophila melanogaster]

    See identical proteins and their annotated locations for NP_609923.2

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q9VJ28
    Related
    FBpp0080728
    Conserved Domains (2) summary
    PRK11728
    Location:56 → 447
    PRK11728; hydroxyglutarate oxidase; Provisional
    cl21454
    Location:69 → 440
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  2. NM_001299148.1 → NP_001286077.1  L-2-hydroxyglutarate dehydrogenase, isoform C [Drosophila melanogaster]

    See identical proteins and their annotated locations for NP_001286077.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q9VJ28
    Conserved Domains (2) summary
    PRK11728
    Location:56 → 447
    PRK11728; hydroxyglutarate oxidase; Provisional
    cl21454
    Location:69 → 440
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  3. NM_001299147.1 → NP_001286076.1  L-2-hydroxyglutarate dehydrogenase, isoform B [Drosophila melanogaster]

    See identical proteins and their annotated locations for NP_001286076.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q9VJ28
    Conserved Domains (2) summary
    PRK11728
    Location:56 → 447
    PRK11728; hydroxyglutarate oxidase; Provisional
    cl21454
    Location:69 → 440
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q95SY9 Q95SY9
Q9VJ28 Q9VJ28

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