Gene/Proteome Database (LMPD)

LMPD ID
LMP004382
Gene ID
12040
Species
Mus musculus (Mouse)
Gene Name
branched chain ketoacid dehydrogenase E1, beta polypeptide
Gene Symbol
Bckdhb
Synonyms
-
Alternate Names
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKDE1B; BCKDH E1-beta; branched-chain alpha-keto acid dehydrogenase E1 component beta chain
Chromosome
9
Map Location
9 E2|9
EC Number
1.2.4.4

Proteins

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Refseq ID NP_954665
Protein GI 40353220
UniProt ID Q6P3A8
mRNA ID NM_199195
Length 322
RefSeq Status PROVISIONAL
MNLFQSITSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRAPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAVEQVPVEPYKIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAQEKLGVSCEVIDLRTIVPWDVDTVCKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY

Gene Information

Entrez Gene ID
12040
Gene Name
branched chain ketoacid dehydrogenase E1, beta polypeptide
Gene Symbol
Bckdhb
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005947 ISS:HGNC C mitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005743 TAS:MGI C mitochondrial inner membrane
GO:0005739 ISS:HGNC C mitochondrion
GO:0003863 IEA:UniProtKB-EC F 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
GO:0003826 IDA:MGI F alpha-ketoacid dehydrogenase activity
GO:0009083 ISS:HGNC P branched-chain amino acid catabolic process
GO:0009063 TAS:MGI P cellular amino acid catabolic process

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY3DJ-6 Leucine Catabolism
PWY3DJ-0 isoleucine degradation

REACTOME Pathway Links

REACTOME Pathway ID Description
5892792 Branched-chain amino acid catabolism

Domain Information

InterPro Annotations

Accession Description
IPR029061 Thiamin diphosphate-binding fold
IPR005476 Transketolase, C-terminal
IPR009014 Transketolase, C-terminal/Pyruvate-ferredoxin oxidoreductase, domain II
IPR005475 Transketolase-like, pyrimidine-binding domain

UniProt Annotations

Entry Information

Gene Name
branched chain ketoacid dehydrogenase E1, beta polypeptide
Protein Entry
ODBB_MOUSE
UniProt ID
Q6P3A8
Species
Mouse

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q6P3A8-1; Sequence=Displayed; Name=2; IsoId=Q6P3A8-2; Sequence=VSP_029841; Note=No experimental confirmation available.;
Catalytic Activity 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2- methylpropanoyl)transferase] S-(2- methylpropanoyl)dihydrolipoyllysine + CO(2).
Function The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Subcellular Location Mitochondrion matrix.
Subunit Heterotetramer of 2 alpha and 2 beta chains. {ECO:0000250}.

Identical and Related Proteins

Unique RefSeq proteins for LMP004382 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
40353220 RefSeq NP_954665 322 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

Identical Sequences to LMP004382 proteins

Reference Database Accession Length Protein Name
GI:40353220 GenBank AAH64099.1 322 Branched chain ketoacid dehydrogenase E1, beta polypeptide [Mus musculus]

Related Sequences to LMP004382 proteins

Reference Database Accession Length Protein Name
GI:40353220 GenBank EDL77651.1 390 branched chain keto acid dehydrogenase E1, beta polypeptide [Rattus norvegicus]
GI:40353220 GenBank AGU32431.1 390 Sequence 118 from patent US 8507277
GI:40353220 PIR - 390 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) (EC 1.2.4.4) - mouse [Mus musculus]
GI:40353220 RefSeq NP_062140.1 390 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial precursor [Rattus norvegicus]
GI:40353220 RefSeq XP_006510848.1 390 PREDICTED: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X1 [Mus musculus]
GI:40353220 SwissProt P35738.3 390 RecName: Full=2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; Short=BCKDE1B; Short=BCKDH E1-beta; Flags: Precursor [Rattus norvegicus]