Rat Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Protein
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Description
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase Protein is a recombinant Rat protein expressed in E. coli.
Documents del producto
Product specifications
| Category | Proteins and Peptides |
| Immunogen Target | Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) |
| Host | E. coli |
| Origin | Rat |
| Conjugation | Unconjugated |
| Observed MW | Molecular Weight: Calculated MW: 24.5 kDa Concentration: Prior to lyophilization: 200 µg/ml Sequence Fragment: Arg44-Thr223 Tag: N-terminal His tag |
| Expression | Recombinant |
| Purity | > 95% |
| Size 1 | 10 µg |
| Size 2 | 50 µg |
| Size 3 | 100 µg |
| Size 4 | 200 µg |
| Size 5 | 500 µg |
| Form | Lyophilized To keep the original salt concentration, we recommend reconstituting to the original concentration prior to lyophilization (see Concentration) in ddH2O. If a lower concentration is required, dilute in PBS, pH 7.4. If a higher concentration is required, the product can be reconstituted directly in PBS, pH 7.4, though please note that this will change the overall salt concentration. The stock concentration should be between 0.1-1.0 mg/ml. Do not vortex. |
| Tested Applications | WB, SDS-PAGE |
| Buffer | Prior to lyophilization: PBS, pH 7.4, containing 0.01% Sarcosyl, 1 mM DTT, 5% Trehalose and Proclin-300. |
| Availability | Shipped within 5-7 working days. |
| Storage | Store at 2-8 °C for up to one month. Store at -80 °C for up to one year. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| Alias | BCKDKD,BCKDH kinase 1,BCKDHKIN,BDK |
| Background | Protein BCKDK |
| Status | RUO |
| Note | This product is for research use only. Not for human consumption, cosmetic, therapeutic or diagnostic use. |
Descripción
Branched Chain Keto Acid Dehydrogenase Kinase (BCKDK) is a pivotal regulatory enzyme in the catabolism of branched-chain amino acids (BCAAs), including leucine, isoleucine, and valine, which are essential amino acids important for protein synthesis, energy production, and metabolic signaling. BCKDK functions by phosphorylating and inhibiting the activity of the branched-chain α-keto acid dehydrogenase (BCKD) complex, a key enzymatic complex responsible for the oxidative decarboxylation of branched-chain α-keto acids derived from BCAAs. This regulatory action of BCKDK prevents excessive degradation of BCAAs and maintains metabolic balance, particularly under conditions requiring the conservation of these amino acids. Dysregulation of BCKDK activity has been implicated in metabolic disorders such as maple syrup urine disease (MSUD), characterized by the accumulation of toxic levels of BCAAs and their corresponding keto acids. Additionally, mutations in the BCKDK gene have been linked to neurodevelopmental disorders and autism spectrum disorders due to altered amino acid metabolism. Given its critical role in amino acid homeostasis and metabolic signaling, BCKDK is a potential therapeutic target for treating metabolic diseases and conditions associated with BCAA dysregulation.
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The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitiously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
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