Thermo Fisher Scientific BCKDK Polyclonal Antibody
BCKDK 단백질을 인식하는 Thermo Fisher의 Rabbit Polyclonal 항체. Western blot, IHC(P), IP에 사용 가능하며, 인간, 마우스, 랫트에 반응. 고순도(>95%) 항원 친화 크로마토그래피 정제. 연구용 전용 제품.
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Applications and Tested Dilutions
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:1,000 |
| Immunohistochemistry (Paraffin) (IHC (P)) | 1:50–1:200 |
| Immunoprecipitation (IP) | 1:50–1:100 |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Synthetic peptide corresponding to amino acids 21–70 of Human BCKDK |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage buffer | PBS, pH 7.2, with 50% glycerol |
| Contains | 0.02% sodium azide |
| 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_2803149 |
Product Specific Information
Purity is >95% by SDS-PAGE.
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.
Clinical relevance: 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 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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