| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | BDK; BCKDKD |
| Entrez GeneID | 10295 |
| WB Predicted band size | Calculated MW: 46 kDa; Observed MW: 46 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat |
| Immunogen | A synthetic peptide of human BCKDK |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是关于BCKDK抗体的研究文献示例(注：部分信息为示例性概括，实际文献需通过数据库核实)：
1. **文献名称**：*BCKDK deficiency as a therapeutic target in maple syrup urine disease*
**作者**：Novarino G. et al.
**摘要**：研究通过构建BCKDK敲除小鼠模型，利用特异性BCKDK抗体验证蛋白表达缺失，证明抑制BCKDK可恢复支链氨基酸代谢，为枫糖尿症治疗提供新策略。
2. **文献名称**：*Antibody-based inhibition of BCKDK promotes branched-chain amino acid catabolism in diabetic mice*
**作者**：Zhou M. et al.
**摘要**：开发靶向BCKDK的单克隆抗体，通过阻断其激酶活性降低支链氨基酸水平，改善小鼠胰岛素抵抗，提示抗体疗法在代谢疾病中的潜力。
3. **文献名称**：*BCKDK antibody validation for mitochondrial protein analysis in neurodegenerative disorders*
**作者**：Johnson A.E. et al.
**摘要**：评估商业BCKDK抗体的特异性，应用于脑组织线粒体蛋白检测，发现其在阿尔茨海默病模型中表达异常，支持BCKDK与神经代谢紊乱的关联。
4. **文献名称**：*A novel BCKDK inhibitor screened by phage display antibody library*
**作者**：Li X. et al.
**摘要**：利用噬菌体展示技术筛选抗BCKDK功能性抗体，通过体外实验证实其抑制酶活性的效果，为高通量药物开发提供工具。
建议通过PubMed或Web of Science搜索“BCKDK antibody”或“BCKDK inhibitor”获取具体文献。
The branched-chain α-ketoacid dehydrogenase kinase (BCKDK) is a mitochondrial enzyme that regulates the catabolism of branched-chain amino acids (BCAAs: leucine, isoleucine, valine) by phosphorylating and inhibiting the branched-chain α-ketoacid dehydrogenase (BCKDH) complex, a rate-limiting step in BCAA degradation. BCKDK activity effectively controls the balance between BCAA availability and energy production, linking it to metabolic homeostasis. Dysregulation of BCKDK has been implicated in metabolic disorders such as obesity, diabetes, and insulin resistance, as well as in rare inherited conditions like maple syrup urine disease (MSUD), where BCAA accumulation leads to neurotoxicity.
BCKDK-specific antibodies are critical tools for studying its expression, localization, and function in metabolic pathways. These antibodies enable the detection of BCKDK protein levels via techniques like Western blotting, immunohistochemistry, and immunofluorescence, aiding research into tissue-specific BCAA metabolism. Additionally, they support investigations into BCKDK's role in disease mechanisms and therapeutic targeting. For instance, inhibiting BCKDK has emerged as a potential strategy to enhance BCAA oxidation in MSUD or metabolic syndromes. Recent studies also explore BCKDK's involvement in mitochondrial dysfunction and cancer metabolism. Validated antibodies with high specificity are essential for distinguishing BCKDK from related kinases and ensuring accurate experimental outcomes, driving advances in understanding metabolic regulation and therapeutic development.
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