Thermo Fisher Scientific BCKDK Polyclonal Antibody
Human, Mouse, Rat에 반응하는 BCKDK 폴리클로날 항체로 Western blot 및 IHC(P) 검증 완료. 항원 친화 크로마토그래피로 정제된 비결합 항체이며, PBS/glycerol buffer에 보관. 연구용으로 BCKDK 단백질 발현 및 기능 연구에 적합.
- 판매단위
- pk
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Applications
| Application | Tested Dilution | Publications |
|---|---|---|
| Western Blot (WB) | 0.04–0.4 µg/mL | - |
| Immunohistochemistry (Paraffin) (IHC (P)) | 1:50–1:200 | - |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant protein corresponding to Human BCKDK (Recombinant protein control fragment: Product #RP-91406) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.3 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage Buffer | PBS, pH 7.2, with 40% 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_2638505 |
Product Specific Information
Immunogen sequence:
LLDDHKDVVT LLAEGLRESR KHIEDEKLVR YFLDKTLTSR LGIRMLATHH LALHEDKPDF VGIICTRLSP KKIIEKWVDF ARRLCEHKYG NAPRVRINGH VAARFPFIPM PLDY
Highest antigen sequence identity to the following orthologs:
- Mouse: 99%
- Rat: 100%
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. BCKDK, which contains one histidine kinase domain, regulates the activity of the BCKD complex.
Maple syrup urine disease (MSUD) is caused by defects in this catabolic pathway, leading to the accumulation of branched-chain amino acids and their 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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