BckdhA Branched chain keto acid dehydrogenase E1 subunit alpha [Drosophila melanogaster (fruit fly)] - Gene

Summary

Official Symbol: BckdhAprovided by FlyBase
Official Full Name: Branched chain keto acid dehydrogenase E1 subunit alphaprovided by FlyBase
Primary source: FLYBASE:FBgn0037709
Locus tag: Dmel_CG8199
See related: AllianceGenome:FB:FBgn0037709
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: bckdhA; CG8199; Dmel\CG8199
Summary: Predicted to enable oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor. Predicted to contribute to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity and branched-chain alpha-keto acid dehydrogenase activity. Predicted to be involved in branched-chain amino acid catabolic process. Predicted to be part of branched-chain alpha-ketoacid dehydrogenase complex and oxoglutarate dehydrogenase complex. Is expressed in several structures, including adult head; adult heart; gut section; and larval muscle system. Used to study maple syrup urine disease. 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]
Orthologs: all
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Genomic context

Location:: 85D24-85D24; 3-48 cM

Exon count:: 4

Annotation release	Status	Assembly	Chr	Location
Release 6.54	current	Release 6 plus ISO1 MT (GCF_000001215.4)	3R	NT_033777.3 (9555785..9557776)
Release 5.57	previous assembly	Release 5 (GCF_000001215.2)	3R	NT_033777.2 (5381507..5383498)

Chromosome 3R - NT_033777.3 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Bibliography

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GeneRIFs: Gene References Into Functions

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Homology

NCBI Orthologs

Orthologs from OrthoDB

Gene Ontology Provided by FlyBase

Function	Evidence Code	Pubs
contributes_to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity	ISS Inferred from Sequence or Structural Similarity more info
contributes_to branched-chain alpha-keto acid dehydrogenase activity	ISS Inferred from Sequence or Structural Similarity 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 branched-chain amino acid catabolic process	ISS Inferred from Sequence or Structural Similarity more info

Component	Evidence Code	Pubs
part_of branched-chain alpha-ketoacid dehydrogenase complex	ISS Inferred from Sequence or Structural Similarity 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: branched chain keto acid dehydrogenase E1 subunit alpha

Names: BckdhA-PA; CG8199-PA; branched-chain a-keto acid dehydrogenase

NP_649905.1

EC 1.2.4.-

EC 1.2.4.4

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

NT_033777.3 Reference assembly

Range

9555785..9557776

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_141648.3 → NP_649905.1 branched chain keto acid dehydrogenase E1 subunit alpha [Drosophila melanogaster]

See identical proteins and their annotated locations for NP_649905.1

Status: REVIEWED

UniProtKB/TrEMBL

Q9VHB8

Related

FBpp0081545

Conserved Domains (1) summary

cd02000
Location:97 → 389

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 ...