全部产品分类
品牌 |
Leading Biology | 货号 |
APG02205G |
产品分类 |
Polyclonal Antibodies | 研究领域 |
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产品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality BCKDK antibody (C-term T340).
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分子量 |
46360 Da
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细胞定位 |
Antigen Cellular Localization:
Mitochondrion matrix. Mitochondrion
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宿主 |
Rabbit
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种属反应性 |
Human, Mouse
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免疫原 |
325-356 aa
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靶点 |
This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 325-356 amino acids from the C-terminal region of human BCKDK.
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亚型 |
Rabbit Ig
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基因ID |
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UniProt ID |
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功能 |
Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.
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总结 |
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 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitiously expressed kinase contains 1 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.
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形式 |
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
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储存条件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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应用 |
WB, E
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稀释方法 |
WB~~1:1000
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别名 |
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
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图像 |
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说明书 |
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数量 |
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