Thermo Fisher Scientific BCKDK Monoclonal Antibody (OTI11C9), TrueMAB
인간 BCKDK 단백질을 인식하는 Mouse IgG1 단일클론 항체로, WB, IHC, ICC/IF에 사용 가능. 고순도 친화 크로마토그래피 정제, 액상 형태로 제공. 연구용으로만 사용되며 -20°C에서 보관.
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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 | 0.8 mg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | PBS 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) |
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 the activity of the BCKD complex: a kinase (BCKDK) and a phosphorylase.
BCKDK is a ubiquitously expressed kinase containing one histidine kinase domain.
Maple syrup urine disease (MSUD) is caused by a defect in the catabolic pathway of leucine, isoleucine, and valine, leading to accumulation of these amino acids and their corresponding keto acids, resulting in encephalopathy and progressive neurodegeneration in infants if untreated.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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