Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
292.5€ (80 µl)
Por favor contáctenos para obtener información detallada sobre el precio y disponibilidad.
935106861
info@markelab.com
name
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx033187
tested applications
ELISA, WB, IHC
Description
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.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | WB: 1/1000, IHC-P: 1/50 - 1/100. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified Rabbit Polyclonal Antibody. |
| Size 1 | 80 µl |
| Size 2 | 400 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IHC |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | O14874 |
| Alias | BCKDKD,BCKDH kinase 1,BCKDHKIN,BDK |
| Background | Antibody anti-BCKDK |
| Status | RUO |
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.
Related Products
Rat Bckdk ([3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial) ELISA Kit
Ver Producto
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
BCKDK Antibody is a Rabbit Polyclonal Antibody against BCKDK.
Ver Producto
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (Bckdk) Antibody
Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex (By similarity).
Ver Producto