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

Gene ID: 172539, updated on 4-Jan-2025

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, Jan 2025]
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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
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