Thermo Fisher Scientific BCKDK Polyclonal Antibody
BCKDK 단백질을 인식하는 Rabbit Polyclonal Antibody로, Human, Mouse, Rat에 반응합니다. Western blot과 Immunocytochemistry에 적합하며, PBS/glycerol buffer에 보관됩니다. Affinity chromatography로 정제된 고품질 항체입니다.
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:3,000 |
| Immunocytochemistry (ICC/IF) | 1:100–1:500 |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | A synthesized peptide derived from human BCKDK (Accession O14874), corresponding to amino acid residues L21–T71 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Affinity chromatography |
| Storage buffer | PBS, pH 7.4, with 50% glycerol |
| Contains | 0.02% sodium azide |
| Storage conditions | −20°C |
| Shipping conditions | Wet ice |
| RRID | AB_2853777 |
Product Specific Information
Antibody detects endogenous levels of total BCKD.
Target Information
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 three catalytic components:
- Branched-chain alpha-keto acid decarboxylase (E1)
- Dihydrolipoyl transacylase (E2)
- Dihydrolipoamide dehydrogenase (E3)
The complex also contains two enzymes that regulate its activity state: a kinase (BCKDK) and a phosphorylase.
The ubiquitously expressed kinase contains one histidine kinase domain.
Maple syrup urine disease (MSUD) is caused by a defect in this catabolic pathway, leading to accumulation of branched-chain amino acids and their corresponding keto acids, resulting in encephalopathy and progressive neurodegeneration in untreated infants.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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