Gene/Proteome Database (LMPD)

LMPD ID
LMP012627
Gene ID
29711
Species
Rattus norvegicus (Rat)
Gene Name
branched chain keto acid dehydrogenase E1, beta polypeptide
Gene Symbol
Bckdhb
Alternate Names
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKDE1B; BCKDH E1-beta; 2-oxoisovalerate dehydrogenase beta subunit; branched chain keto acid dehydrogenase E1 beta; branched-chain alpha-keto acid dehydrogenase E1 component beta chain;
Chromosome
8
Map Location
8q31
EC Number
1.2.4.4
Summary
plays a role in the conversion of 3-methyl-2-oxobutanoate and lipoamide to S-(2-methylpropanoyl)dihydrolipoamide and CO2 [RGD, Feb 2006]
Orthologs
View orthologs and multiple alignments for Bckdhb

Proteins

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial precursor
Refseq ID NP_062140
Protein GI 158749538
UniProt ID P35738
mRNA ID NM_019267
Length 390
MAAVAARAGGLLRLGAAGAERRRRGLRCAALVQGFLQPAVDDASQKRRVAHFTFQPDPESLQYGQTQKMNLFQSITSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRAPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAVEQVPVEPYKIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAQEKLGVSCEVIDLRTIVPWDVDTVCKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
transit_peptide: 1..48 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (Potential); propagated from UniProtKB/Swiss-Prot (P35738.3) calculated_mol_wt: 5033 peptide sequence: MAAVAARAGGLLRLGAAGAERRRRGLRCAALVQGFLQPAVDDASQKRR mat_peptide: 49..390 product: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P35738.3) calculated_mol_wt: 37809 peptide sequence: VAHFTFQPDPESLQYGQTQKMNLFQSITSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRAPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAVEQVPVEPYKIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAQEKLGVSCEVIDLRTIVPWDVDTVCKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY

Gene Information

Entrez Gene ID
29711
Gene Name
branched chain keto acid dehydrogenase E1, beta polypeptide
Gene Symbol
Bckdhb
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005947 ISS:HGNC C mitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005739 ISS:HGNC C mitochondrion
GO:0003863 IEA:UniProtKB-EC F 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
GO:0003826 IEA:Ensembl F alpha-ketoacid dehydrogenase activity
GO:0032403 IPI:RGD F protein complex binding
GO:0009083 ISS:HGNC P branched-chain amino acid catabolic process
GO:0051591 IEP:RGD P response to cAMP
GO:0051384 IEP:RGD P response to glucocorticoid
GO:0007584 IEP:RGD P response to nutrient

KEGG Pathway Links

KEGG Pathway ID Description
M00036 Leucine degradation, leucine => acetoacetate + acetyl-CoA
rno01100 Metabolic pathways
ko00280 Valine, leucine and isoleucine degradation
rno00280 Valine, leucine and isoleucine degradation

REACTOME Pathway Links

REACTOME Pathway ID Description
5953282 Branched-chain amino acid catabolism
5953250 Metabolism
5953272 Metabolism of amino acids and derivatives

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 keto acid dehydrogenase E1, beta polypeptide
Protein Entry
ODBB_RAT
UniProt ID
P35738
Species
Rat

Comments

Comment Type Description
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).
Sequence Caution Sequence=AAA73899.1; Type=Frameshift; Positions=101, 120; Evidence= ; Sequence=AAA73899.1; Type=Miscellaneous discrepancy; Note=Contaminating sequence. Sequence of unknown origin in the N-terminal part.; Evidence= ;
Subcellular Location Mitochondrion matrix.
Subunit Heterotetramer of 2 alpha and 2 beta chains

Identical and Related Proteins

Unique RefSeq proteins for LMP012627 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
158749538 RefSeq NP_062140 390 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial precursor

Identical Sequences to LMP012627 proteins

Reference Database Accession Length Protein Name
GI:158749538 GenBank EDL77651.1 390 branched chain keto acid dehydrogenase E1, beta polypeptide [Rattus norvegicus]
GI:158749538 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]

Related Sequences to LMP012627 proteins

Reference Database Accession Length Protein Name
GI:158749538 GenBank EDL77651.1 390 branched chain keto acid dehydrogenase E1, beta polypeptide [Rattus norvegicus]
GI:158749538 RefSeq XP_006243512.1 405 PREDICTED: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X1 [Rattus norvegicus]
GI:158749538 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]