Thermo Fisher Scientific BCKDH Polyclonal Antibody, Biotin
인간, 마우스, 랫트 반응성을 가진 BCKDH 폴리클로날 항체로, 비오틴 결합 형태입니다. Western blot, ELISA, Immunoprecipitation에 적합하며, 합성 펩타이드 면역원으로 제작되었습니다. 친화 크로마토그래피로 정제되어 고순도, 안정적입니다.
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Thermo Fisher Scientific BCKDH Polyclonal Antibody, Biotin
Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:1,000 |
| ELISA | 1:10,000 |
| Immunoprecipitation (IP) | 1:50–1:250 |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Synthetic peptide taken within amino acid region 170–200 on human BCKD protein |
| Conjugate | Biotin |
| Form | Liquid |
| Concentration | 0.5–1.5 mg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | Proprietary buffer, pH 7.4–7.8, with 0.5% BSA, 30% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | –20°C |
| 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).
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 regulatory enzymes:
- Kinase (BCKDK)
- Phosphorylase
The ubiquitously expressed kinase contains one histidine kinase domain.
Defects in this pathway lead to Maple Syrup Urine Disease (MSUD), characterized by the 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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