bckd-1A 2-oxoisovalerate dehydrogenase subunit alpha;2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: bckd-1A
Official Full Name: 2-oxoisovalerate dehydrogenase subunit alpha;2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Primary source: WormBase:WBGene00012713
Locus tag: CELE_Y39E4A.3
See related: AllianceGenome:WB:WBGene00012713
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 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity and metal ion binding activity. Predicted to be involved in branched-chain amino acid catabolic process. Predicted to be located in mitochondrial matrix. Predicted to be part of oxoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha). [provided by Alliance of Genome Resources, Dec 2024]
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Genomic context

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See bckd-1A in Genome Data Viewer

Location:: chromosome: III

Exon count:: 5

Sequence:: Chromosome: III; NC_003281.10 (12967937..12972498, complement)

Chromosome III - NC_003281.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003281.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

TK can catalyze the single-substrate reaction of xylulose-5-phosphate in the absence of the acceptor substrate, ribose 5-phosphate.
Title: Isolation and properties of human transketolase.

Submit: New GeneRIF Correction

Pathways from PubChem

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

Y39E4A.3 (e-PCR)
- UniSTS:319759

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor	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 fatty acid biosynthetic process	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
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: 2-oxoisovalerate dehydrogenase subunit alpha;2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

NP_001033376.1

Confirmed by transcript evidence

NP_001033377.1

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

NC_003281.10 Reference assembly

Range

12967937..12972498 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_001038287.6 → NP_001033376.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_001033376.1

Status: REVIEWED

UniProtKB/Swiss-Prot

O45924

Conserved Domains (1) summary

cd02000
Location:88 → 380

TPP_E1_PDC_ADC_BCADC; Thiamine pyrophosphate (TPP) family, E1 of PDC_ADC_BCADC subfamily, TPP-binding module; composed of proteins similar to the E1 components of the human pyruvate dehydrogenase complex (PDC), the acetoin dehydrogenase complex (ADC) and the branched chain ...
NM_001038288.6 → NP_001033377.1 2-oxoisovalerate dehydrogenase subunit alpha [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_001033377.1

Status: REVIEWED

UniProtKB/TrEMBL

Q4A1S8

Conserved Domains (1) summary

cd02000
Location:89 → 381

TPP_E1_PDC_ADC_BCADC; Thiamine pyrophosphate (TPP) family, E1 of PDC_ADC_BCADC subfamily, TPP-binding module; composed of proteins similar to the E1 components of the human pyruvate dehydrogenase complex (PDC), the acetoin dehydrogenase complex (ADC) and the branched chain ...