Thermo Fisher Scientific BCKDK Recombinant Rabbit Monoclonal Antibody (JE63-77)
BCKDK 단백질을 인식하는 토끼 유래 재조합 단일클론 항체로, Western blot에 적합합니다. 인간 시료에 반응하며 HEK293 세포에서 발현되었습니다. 고순도 Protein A 정제, 액상 형태로 제공되며 안정적 장기 보관이 가능합니다.
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Applications
- Western Blot (WB): Tested dilution 1:500
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Expression System | HEK293 cells |
| Class | Recombinant Monoclonal |
| Type | Antibody |
| Clone | JE63-77 |
| Immunogen | Synthetic peptide within human BCKDK aa 50–100/412 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Protein A |
| Storage Buffer | TBS, pH 7.4, with 40% glycerol, 0.05% BSA |
| Contains | 0.05% sodium azide |
| Storage Conditions | Store at 4°C short term. For long term, store at -20°C, avoiding freeze/thaw cycles. |
| Shipping Conditions | Wet ice |
| RRID | AB_2931233 |
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 is 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.
BCKDK is ubiquitously expressed and contains one histidine kinase domain.
Defects in this catabolic pathway lead to Maple Syrup Urine Disease (MSUD), characterized by accumulation of branched-chain amino acids and keto acids, resulting in 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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