MitoProteome Database

MT000767

Record overview

MITO ID	MT000767
Gene ID	10295
Species	Homo sapiens (Human)
Gene Name	branched chain ketoacid dehydrogenase kinase
Gene Symbol	BCKDK
Synonyms	BDK; BCKDKD;
Alternate names	[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKDHKIN; BCKD-kinase; branched chain alpha-ketoacid dehydrogenase kinase;
Chromosome	16
Map Location	16p11.2
EC Number	2.7.11.4
Summary	The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]
Orthologs	View orthologs and multiple alignments for BCKDK

Proteins

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform a precursor

Refseq ID:	NP_005872
Protein GI:	171906589
UniProt ID:	O14874
mRNA ID:	NM_005881
Length:	412
RefSeq Status:

MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPV
RIAHRIKGFRCLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGI
RMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVI
TIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRL
RHIDGREESFRI

N
transit_peptide: 1..30
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O14874.2)
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

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 (O14874.2)
calculated_mol_wt: 43355
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMD
YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform b precursor

Refseq ID:	NP_001116429
Protein GI:	171906593
UniProt ID:	O14874
mRNA ID:	NM_001122957
Length:	365
RefSeq Status:

`MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPV RIAHRIKGFRCLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGI RMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVI TIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHG`

N
transit_peptide: 1..30
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O14874.2)
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

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 (O14874.2)
calculated_mol_wt: 43355
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMD
YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI

transit_peptide: 1..30
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O14874.2)
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

mat_peptide: 31..365
product: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform b
calculated_mol_wt: 38084
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMD
YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHG

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform c

Refseq ID:	NP_001258855
Protein GI:	429484478
UniProt ID:	O14874
mRNA ID:	NM_001271926
Length:	335
RefSeq Status:

`MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPV RIAHRIKGFRCLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGI RMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRISDRGGGIAHKDLDRVMD YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHG`

N
transit_peptide: 1..30
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O14874.2)
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

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 (O14874.2)
calculated_mol_wt: 43355
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMD
YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI

transit_peptide: 1..30
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (O14874.2)
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

mat_peptide: 31..365
product: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform b
calculated_mol_wt: 38084
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMD
YHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHG

transit_peptide: 1..30
calculated_mol_wt: 3024
peptide sequence: 
MILASVLRSGPGGGLPLRPLLGPALALRAR

mat_peptide: 31..335
product: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial isoform c
calculated_mol_wt: 34762
peptide sequence: 
STSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYI
RAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKI
IEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQS
GPMHG