Thermo Fisher Scientific BCKDK Recombinant Rabbit Monoclonal Antibody (5J3L3)
BCKDK 단백질을 인식하는 토끼 유래 리콤비넌트 단클론 항체로, Western blot 및 ELISA에 적합합니다. HEK293 세포에서 발현되었으며 Protein A로 정제되었습니다. 인간, 마우스, 랫트 시료에 반응하며 -20°C에서 보관합니다.
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:2,000 |
| ELISA | 1 µg/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Expression System | HEK293 cells |
| Class | Recombinant Monoclonal |
| Type | Antibody |
| Clone | 5J3L3 |
| Immunogen | A synthetic peptide corresponding to amino acids 1–100 of human BCKDK |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.5 mg/mL |
| Purification | Protein A |
| Storage Buffer | PBS, pH 7.3, with 0.05% BSA, 50% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
Product Specific Information
Immunogen Sequence:
MILASVLRSG PGGGLPLRPL LGPALALRAR STSATDTHHV EMARERSKTV TSFYNQSAID AAAEKPSVRL TPTMMLYAGR SQDGSHLLKS ARYLQQELPV
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 the activity state of the BCKD complex: a kinase (BCKDK) and a phosphorylase. The ubiquitously expressed kinase contains one histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology resulting from an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine, leading to accumulation of these amino acids and their corresponding 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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