Thermo Fisher Scientific BCKDK Polyclonal Antibody
BCKDK 단백질을 인식하는 Thermo Fisher Scientific의 폴리클로날 항체로, WB 및 ICC/IF에 적합. 인간, 생쥐, 랫트 반응성. 항원 친화 크로마토그래피로 정제된 액상 형태. 연구용으로만 사용 가능.
- 카탈로그번호
- PA549761
- 판매단위
- pk
카탈로그
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Applications
Western Blot (WB)
- Tested Dilution: 1:500–1:3,000
Immunocytochemistry (ICC/IF)
- Tested Dilution: 1:100–1:500
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Synthesized peptide derived from internal region of human BCKD (aa 32–58) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Antigen 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_2635214 |
Product Specific Information
The antibody detects endogenous levels of total BCKDK protein.
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) arises from a defect in this catabolic pathway, leading to accumulation of branched-chain amino acids and their 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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