Thermo Fisher Scientific BCKDK Polyclonal Antibody
Thermo Fisher Scientific의 BCKDK Polyclonal Antibody는 인간, 마우스, 랫트에 반응하는 Rabbit IgG 항체로, WB, IHC, ICC/IF에 사용 가능. 항원 친화 크로마토그래피로 정제되어 높은 특이성과 안정성을 제공하며, 연구용으로 적합.
- 판매단위
- pk
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Applications
Western Blot (WB)
- Tested Dilution: 1:500–1:20,000
Immunohistochemistry (Paraffin) (IHC (P))
- Tested Dilution: 1:100–1:1,000
Immunocytochemistry (ICC/IF)
- Tested Dilution: Assay-dependent
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant fragment corresponding to a region within amino acids 77–367 of Human BCKDK |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.35 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage Buffer | PBS, pH 7, with 20% glycerol, 1% BSA |
| Contains | 0.025% ProClin 300 |
| Storage Conditions | Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
| Shipping Conditions | Wet ice |
| RRID | AB_2545526 |
Product Specific Information
- Recommended positive controls: MCF-7, MCF-7 membrane extract, Mouse brain, Rat brain, Rat kidney
- Predicted reactivity: Mouse (99%), Rat (100%), Zebrafish (82%), Xenopus laevis (87%), Rhesus Monkey (98%), Bovine (98%)
- Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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