Primary Information |
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| BoMiProt ID | Bomi3431 |
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| Protein Name | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial/Branched-chain alpha-ketoacid dehydrogenase kinase/BCKD-kinase/BCKDHKIN |
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| Organism | Bos taurus |
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| Uniprot ID | Q2KJG8 |
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| Milk Fraction | Whey |
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| Ref Sequence ID | NP_001039371.1 |
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| Aminoacid Length | 412 |
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| Molecular Weight | 46438 |
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| FASTA Sequence |
Download |
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| Gene Name | BCKDK |
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| Gene ID | 505005 |
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| Protein Existence Status | reviewed |
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Secondary Information |
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| Protein Function | Regulates TCA cycle through PDC in the absence of PDK family during embryonic development.Also regulates catabolism of branched amino acids. |
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| 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. |
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| PTMs | Auto phosphorylated, Acetylation |
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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 |
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| Predicted Disorder Regions | 12-17, 29-44 |
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| DisProt Annotation | |
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| TM Helix Prediction | No TM helices |
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| 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. |
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| Linking IDs | |
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| 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. |
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