Search by BoMiProt ID - Bomi3431


Primary Information

BoMiProt ID Bomi3431
Protein Name [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial/Branched-chain alpha-ketoacid dehydrogenase kinase/BCKD-kinase/BCKDHKIN
Organism Bos taurus
Uniprot IdQ2KJG8
Milk FractionWhey
Ref Sequence Id NP_001039371.1
Aminoacid Length 412
Molecular Weight 46438
Fasta Sequence https://www.uniprot.org/uniprot/Q2KJG8.fasta
Gene Name BCKDK
Gene Id 505005
Protein Existence Status reviewed

Secondary Information

Protein Function Regulates TCA cycle through PDC in the absence of PDK family during embryonic development.Also regulates catabolism of branched amino acids.
Biochemical Properties ATP + L-seryl-[3-methyl-2-oxobutanoate dehydrogenase] = ADP + H+ + O-phospho-L-seryl-[3-methyl-2-oxobutanoate dehydrogenase] BCKDK is a key negative regulation enzyme in BCAA catabolism that inhibits the dehydrogenase activity of the branched-chain α-keto acid dehydrogenase (BCKDH) complex by dephosphorylating the E1 component of the complex.
PTMs Auto phosphorylated, Acetylation
Site(s) of PTM(s)

N-glycosylation, O-glycosylation,
Phosphorylation
>sp|Q2KJG8|BCKD_BOVIN [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial OS=Bos taurus OX=9913 GN=BCKDK PE=2 SV=1 MILASVLGSGPRGGPPLRPLLGPALSLRARS*31TSATDTHHVEMARERSKTVTS*52FYNQSAIDVAAEKPSVRLTPTMMLYSGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRSLPFIIGCNP TILHVHELYIRAFQKLTDFPPIKDQADEARYCQLVRQLLDDHKDVVTLLAEGLRESRKYI EDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARR LCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVI TIANNDIDLVIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDLHS*356GGQS*360 GPMHGFGFGLPTSRAYAEYLGGSLRLQSLQGIGTDVYLRLRHIDGREESFRI
Predicted Disorder Regions 12-17, 29-44
DisProt Annotation
TM Helix Prediction No TM helices
Additional Comments 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.
Linking IDs
Bibliography Heinemann-Yerushalmi L, Bentovim L, Felsenthal N, Vinestock RC, Michaeli N, Krief S, Silberman A, Cohen M, Ben-Dor S, Brenner O, Haffner-Krausz R, Itkin M, Malitsky S, Erez A, Zelzer E. BCKDK regulates the TCA cycle through PDC in the absence of PDK family during embryonic development. Dev Cell. 2021 Apr 19;56(8):1182-1194.e6. doi: 10.1016/j.devcel.2021.03.007. Epub 2021 Mar 26. PMID: 33773101.