Thermo Fisher Scientific BCKDH Polyclonal Antibody
BCKDH 단백질을 인식하는 Rabbit Polyclonal 항체로 Western blot, ELISA, IP에 사용 가능. Human, Mouse, Rat 반응성. 합성 펩타이드(aa 170–200) 면역원 사용. Affinity chromatography로 정제된 액상 항체로 -20°C 보관.
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Applications and Tested Dilutions
| 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 within amino acid region 170–200 on human BCKD protein |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.5–1.5 mg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | Proprietary buffer (pH 7.4–7.8) with 30% glycerol, 0.5% BSA |
| 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), 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 includes two regulatory enzymes: a kinase (BCKDK) and a phosphorylase. The ubiquitously expressed kinase contains one histidine kinase domain. Maple syrup urine disease (MSUD) results from defects in this catabolic pathway, leading to accumulation of branched-chain amino acids and their keto acids, causing 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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