BCKDK Antibody
(Synonyms: BCKDK, Branched Chain Keto Acid Dehydrogenase Kinase, [3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide]] Kinase, Mitochondrial, Branched-Chain Alpha-Ketoacid Dehydrogenase Kinase, BCKDH Kinase, EC 2.7.11.4, BCKDHKIN, BDK, 3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide] Kinase, Mitochondrial, Branched Chain Alpha-Ketoacid Dehydrogenase Kinase, Branched Chain Ketoacid Dehydrogenase Kinase, BCKD-Kinase, EC 2.7.11.1, EC 2.7.11, BCKDKD)BCKDK Antibody is a Rabbit-derived and non-conjugated IgG polyclonal antibody, targeting to BCKDK.
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Host:
Rabbit
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Isotype:
IgG
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Application:
WB, IHC-P
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Reactivity :
Human, mouse, rat
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Formulation:
Supplied in PBS (pH 7.4) containing 50% glycerol, 0.02% sodium azide.
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Conjugation:
Non-conjugated
Applications
| Application |
WB
WB: Western Blot
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IHC-P
IHC-P: Immunohistochemistry-Paraffin
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|---|---|---|
| Dilution Ratio | 1:1000-5000 | 1:100-200 |
Product Details
BCKDK Antibody is a Rabbit-derived and non-conjugated IgG polyclonal antibody, targeting to BCKDK.
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Host Rabbit
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Species ReactivityHuman, mouse, rat
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Observed Molecular WeightObserved band size: 42 kDaNote: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
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Calculated Molecular Weight Predicted band size: 46 kDa,
A synthesized peptide derived from human BCKDK
affinity purified.
Non-conjugated
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in PBS (pH 7.4) containing 50% glycerol, 0.02% sodium azide.
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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Function
BCKDK is a Serine/threonine-protein kinase component of macronutrients metabolism. Forms a functional kinase and phosphatase pair with PPM1K, serving as a metabolic regulatory node that coordinates branched-chain amino acids (BCAAs) with glucose and lipid metabolism via two distinct phosphoprotein targets: mitochondrial BCKDHA subunit of the branched-chain alpha-ketoacid dehydrogenase (BCKDH) complex and cytosolic ACLY, a lipogenic enzyme of Krebs cycle. Phosphorylates and inactivates mitochondrial BCKDH complex a multisubunit complex consisting of three multimeric components each involved in different steps of BCAA catabolism: E1 composed of BCKDHA and BCKDHB, E2 core composed of DBT monomers, and E3 composed of DLD monomers. Associates with the E2 component of BCKDH complex and phosphorylates BCKDHA on Ser-337, leading to conformational changes that interrupt substrate channeling between E1 and E2 and inactivates the BCKDH complex. Phosphorylates ACLY on Ser-455 in response to changes in cellular carbohydrate abundance such as occurs during fasting to feeding metabolic transition. Refeeding stimulates MLXIPL/ChREBP transcription factor, leading to increased BCKDK to PPM1K expression ratio, phosphorylation and activation of ACLY that ultimately results in the generation of malonyl-CoA and oxaloacetate immediate substrates of de novo lipogenesis and glucogenesis, respectively. Recognizes phosphosites having SxxE/D canonical motif[1][2][3].
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Subcellular Localization
Mitochondrion matrix
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Expression
Tissue_Specificity: Ubiquitous. -
Isoforms & Post-Translational Modification
BCKDK has 3 isoforms, O14874-1: amino acid length is 412, molecular weight is 46360 Da (predicted); O14874-2: amino acid length is 335, molecular weight is 37768 Da (predicted); O14874-3: amino acid length is 365, molecular weight is 41090 Da (predicted).可发生自磷酸化BCKDK 存在 3 个异构体,O14874-1:氨基酸个数为 412 个,分子量为 46360 Da (预测);O14874-2:氨基酸个数为 335 个,分子量为 37768 Da (预测);O14874-3:氨基酸个数为 365 个,分子量为 41090 Da (预测)。Autophosphorylated
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Subunit
Homodimer.
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SwissProt ID
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Synonyms
BCKDK, Branched Chain Keto Acid Dehydrogenase Kinase, [3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide]] Kinase, Mitochondrial, Branched-Chain Alpha-Ketoacid Dehydrogenase Kinase, BCKDH Kinase, EC 2.7.11.4, BCKDHKIN, BDK, 3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide] Kinase, Mitochondrial, Branched Chain Alpha-Ketoacid Dehydrogenase Kinase, Branched Chain Ketoacid Dehydrogenase Kinase, BCKD-Kinase, EC 2.7.11.1, EC 2.7.11, BCKDKD
Documentation
[1]. García-Cazorla A, et al. Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients. Hum Mutat. 2014 Apr;35(4):470-7. [Content Brief]
[2]. White PJ, et al. The BCKDH Kinase and Phosphatase Integrate BCAA and Lipid Metabolism via Regulation of ATP-Citrate Lyase. Cell Metab. 2018 Jun 5;27(6):1281-1293.e7. [Content Brief]
[3]. Roth Flach RJ, et al. Small molecule branched-chain ketoacid dehydrogenase kinase (BDK) inhibitors with opposing effects on BDK protein levels. Nat Commun. 2023 Aug 9;14(1):4812. [Content Brief]