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

    Gene ID: 35156, updated on 17-Dec-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
    Enables 2-hydroxyglutarate dehydrogenase activity, forward reaction. Predicted to be located 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, Dec 2024]
    Orthologs
    all
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    Genomic context

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    See L2HGDH in Genome Data Viewer
    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. Structure and biochemical characterization of l-2-hydroxyglutarate dehydrogenase and its role in the pathogenesis of l-2-hydroxyglutaric aciduria. Yang J, et al. J Biol Chem, 2024 Jan. PMID 37995940, Free PMC Article
    2. 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
    3. 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
    4. 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
    5. 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

    See all (33) 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 IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables 2-hydroxyglutarate dehydrogenase activity, forward reaction IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    located_in mitochondrion ISS
    Inferred from Sequence or Structural Similarity
    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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