Gene/Proteome Database (LMPD)

LMPD ID
LMP002817
Gene ID
12041
Species
Mus musculus (Mouse)
Gene Name
branched chain ketoacid dehydrogenase kinase
Gene Symbol
Bckdk
Synonyms
AI327402
Alternate Names
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKDHKIN; BCKD-kinase; branched-chain alpha-ketoacid dehydrogenase kinase
Chromosome
7
Map Location
7 F3|7
EC Number
2.7.11.4

Proteins

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor
Refseq ID NP_033869
Protein GI 6753164
UniProt ID O55028
mRNA ID NM_009739
Length 412
RefSeq Status VALIDATED
MILTSVLGSGPRSWSSLWPLLGSSLSLRARSTSATDTHHVELARERSKTVTSFYNQSAIDVAAEKPSVRLTPTMMLYSGRSQDGSHLLKSGRYLQQELPVRIAHRIKGFRSLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIQDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDIDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRINPLFGHLDMHSGGQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI
transit_peptide: 1..30 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O55028.1) calculated_mol_wt: 3229 peptide sequence: MILTSVLGSGPRSWSSLWPLLGSSLSLRAR mat_peptide: 31..412 product: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (O55028.1) calculated_mol_wt: 43377 peptide sequence: STSATDTHHVELARERSKTVTSFYNQSAIDVAAEKPSVRLTPTMMLYSGRSQDGSHLLKSGRYLQQELPVRIAHRIKGFRSLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIQDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDIDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRINPLFGHLDMHSGGQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI

Gene Information

Entrez Gene ID
12041
Gene Name
branched chain ketoacid dehydrogenase kinase
Gene Symbol
Bckdk
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005947 ISS:HGNC C mitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005739 IDA:MGI C mitochondrion
GO:0005524 ISS:HGNC F ATP binding
GO:0047323 IEA:UniProtKB-EC F [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity
GO:0004672 IDA:MGI F protein kinase activity
GO:0004674 ISS:HGNC F protein serine/threonine kinase activity
GO:0009083 ISS:HGNC P branched-chain amino acid catabolic process
GO:0016310 ISS:HGNC P phosphorylation
GO:0006468 IDA:GOC P protein phosphorylation

Domain Information

InterPro Annotations

Accession Description
IPR018955 Branched-chain alpha-ketoacid dehydrogenase kinase/Pyruvate dehydrogenase kinase, N-terminal
IPR003594 Histidine kinase-like ATPase, C-terminal domain
IPR005467 Signal transduction histidine kinase, core
IPR004358 Signal transduction histidine kinase-related protein, C-terminal

UniProt Annotations

Entry Information

Gene Name
branched chain ketoacid dehydrogenase kinase
Protein Entry
BCKD_MOUSE
UniProt ID
O55028
Species
Mouse

Comments

Comment Type Description
Catalytic Activity ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate.
Disruption Phenotype Mutant animals are born at the expected Mendelian ratio, but they exhibit a decreased fertility. They are healthy at birth. At 3 weeks of age, they start showing growth retardation. At 12 weeks, they are 15% smaller than their wild- type littermates. Adults develop neurological abnormalities, such as tremors, epileptic seizures and hindlimb clasping. These neurological deficits can be completely abolished when mice are fed with a diet enriched in branched amino acids. {ECO:0000269|PubMed:16875466, ECO:0000269|PubMed:22956686}.
Function Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex (By similarity). {ECO:0000250}.
Induction Expression in female liver is influenced by circadian rhythm.
Ptm Autophosphorylated. {ECO:0000250}.
Similarity Belongs to the PDK/BCKDK protein kinase family. {ECO:0000305}.
Similarity Contains 1 histidine kinase domain. {ECO:0000255|PROSITE-ProRule:PRU00107}.
Subcellular Location Mitochondrion matrix.
Subunit Monomer. {ECO:0000250}.
Tissue Specificity Ubiquitous.

Identical and Related Proteins

Unique RefSeq proteins for LMP002817 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
6753164 RefSeq NP_033869 412 [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor

Identical Sequences to LMP002817 proteins

Reference Database Accession Length Protein Name
GI:6753164 DBBJ BAE29698.1 412 unnamed protein product [Mus musculus]
GI:6753164 DBBJ BAE39591.1 412 unnamed protein product [Mus musculus]
GI:6753164 DBBJ BAE26459.1 412 unnamed protein product [Mus musculus]
GI:6753164 GenBank ABK42402.1 412 BCKDK [synthetic construct]
GI:6753164 GenBank EDL17602.1 412 branched chain ketoacid dehydrogenase kinase, isoform CRA_a [Mus musculus]
GI:6753164 GenBank EDL17603.1 412 branched chain ketoacid dehydrogenase kinase, isoform CRA_a [Mus musculus]

Related Sequences to LMP002817 proteins

Reference Database Accession Length Protein Name
GI:6753164 DBBJ BAC35850.1 412 unnamed protein product [Mus musculus]
GI:6753164 DBBJ BAE29801.1 412 unnamed protein product [Mus musculus]
GI:6753164 GenBank EDM17216.1 412 rCG39586, isoform CRA_a [Rattus norvegicus]
GI:6753164 GenBank EDM17217.1 412 rCG39586, isoform CRA_a [Rattus norvegicus]
GI:6753164 RefSeq NP_062117.2 412 [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor [Rattus norvegicus]
GI:6753164 SwissProt Q00972.2 412 RecName: Full=[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; AltName: Full=Branched-chain alpha-ketoacid dehydrogenase kinase; Short=BCKD-kinase; Short=BCKDHKIN; Flags: Precursor [Rattus norvegicus]