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    bckd-1B 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) [ Caenorhabditis elegans ]

    Gene ID: 172539, updated on 9-Dec-2024

    Summary

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    Official Symbol
    bckd-1B
    Official Full Name
    3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
    Primary source
    WormBase:WBGene00006518
    Locus tag
    CELE_F27D4.5
    See related
    AllianceGenome:WB:WBGene00006518
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Caenorhabditis elegans (strain: Bristol N2)
    Lineage
    Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
    Summary
    Predicted to enable catalytic activity. Involved in positive regulation of fatty acid biosynthetic process and positive regulation of nematode larval development. Predicted to be located in mitochondrion. Predicted to be part of oxoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta). [provided by Alliance of Genome Resources, Dec 2024]
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    Genomic context

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    See bckd-1B in Genome Data Viewer
    Location:
    chromosome: I
    Exon count:
    4
    Sequence:
    Chromosome: I; NC_003279.8 (7727267..7729092)

    Chromosome I - NC_003279.8Genomic Context describing neighboring genes Neighboring gene Vesicle transport protein Neighboring gene Zinc finger CCCH domain-containing protein 15 homolog Neighboring gene ncRNA Neighboring gene Chromate transporter Neighboring gene C2H2-type domain-containing protein Neighboring gene ncRNA

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_003279.8

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Developmental Defects of Caenorhabditis elegans Lacking Branched-chain α-Ketoacid Dehydrogenase Are Mainly Caused by Monomethyl Branched-chain Fatty Acid Deficiency. Jia F, et al. J Biol Chem, 2016 Feb 5. PMID 26683372, Free PMC Article
    2. Metformin rescues Parkinson's disease phenotypes caused by hyperactive mitochondria. Mor DE, et al. Proc Natl Acad Sci U S A, 2020 Oct 20. PMID 33024014, Free PMC Article
    3. Pathogenesis of human mitochondrial diseases is modulated by reduced activity of the ubiquitin/proteasome system. Segref A, et al. Cell Metab, 2014 Apr 1. PMID 24703696
    4. Systems-level transcriptional regulation of Caenorhabditis elegans metabolism. Nanda S, et al. Mol Syst Biol, 2023 May 9. PMID 36942755, Free PMC Article
    5. Caenorhabditis elegans ALG-1 antimorphic mutations uncover functions for Argonaute in microRNA guide strand selection and passenger strand disposal. Zinovyeva AY, et al. Proc Natl Acad Sci U S A, 2015 Sep 22. PMID 26351692, Free PMC Article

    See all (16) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Markers

    Homology

    Gene Ontology Provided by WormBase

    Function Evidence Code Pubs
    enables catalytic activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    Process Evidence Code Pubs
    involved_in branched-chain amino acid catabolic process IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in positive regulation of fatty acid biosynthetic process IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in positive regulation of nematode larval development IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in response to nutrient IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    Component Evidence Code Pubs
    located_in mitochondrion IEA
    Inferred from Electronic Annotation
    more info
    		  
    part_of oxoglutarate dehydrogenase complex IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  

    General protein information

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    Preferred Names
    3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
    NP_492149.1
    • Confirmed by transcript evidence

    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. NC_003279.8 Reference assembly

      Range
      7727267..7729092
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_059748.5 → NP_492149.1  3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) [Caenorhabditis elegans]

      See identical proteins and their annotated locations for NP_492149.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q93619
      Conserved Domains (1) summary
      PTZ00182
      Location:6 → 364
      PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional
    Nucleotide Protein
    Heading Accession and Version

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