Thermo Fisher Scientific BCKDK Monoclonal Antibody (OTI11C9)
인간 BCKDK 단백질을 인식하는 Mouse IgG1 단일클론 항체로 Western blot, IHC, ICC/IF에 사용 가능. 고순도 Affinity Chromatography 정제, 1 mg/mL 농도 액상 형태. 연구용으로만 사용, 진단용 불가.
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:2,000 |
| Immunohistochemistry (Paraffin) (IHC (P)) | 1:50 |
| Immunocytochemistry (ICC/IF) | 1:100 |
Product Specifications
| Specification | Description |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Mouse / IgG1 |
| Class | Monoclonal |
| Type | Antibody |
| Clone | OTI11C9 |
| Immunogen | Full length human recombinant protein of BCKDK produced in HEK293T cells |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Affinity Chromatography |
| Storage Buffer | PBS, pH 7.3, with 1% BSA, 50% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
| RRID | AB_2722891 |
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). This inner-mitochondrial enzyme complex consists of three catalytic components:
- Branched-chain alpha-keto acid decarboxylase (E1)
- Dihydrolipoyl transacylase (E2)
- Dihydrolipoamide dehydrogenase (E3)
The complex also includes two regulatory enzymes: a kinase (BCKDK) and a phosphorylase.
BCKDK, which contains one histidine kinase domain, is ubiquitously expressed.
Defects in this catabolic pathway lead to Maple Syrup Urine Disease (MSUD), characterized by accumulation of branched-chain amino acids and their keto acids, resulting in encephalopathy and neurodegeneration in untreated infants.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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