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安全信息

HPA017995

Sigma-Aldrich

Anti-BCKDK antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

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别名:
Anti-BCKD-kinase, Anti-BCKDHKIN, Anti-Branched-chain alpha-ketoacid dehydrogenase kinase, Anti-[3-Methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor
UNSPSC代码:
12352203
人类蛋白质图谱编号:
NACRES:
NA.41

生物来源

rabbit

质量水平

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

产品线

Prestige Antibodies® Powered by Atlas Antibodies

形式

buffered aqueous glycerol solution

种属反应性

mouse, human, rat

技术

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:50-1:200

免疫原序列

LLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDY

UniProt登记号

运输

wet ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... BCKDK(10295)

一般描述

Branched chain ketoacid dehydrogenase kinase (BCKDK) is highly expressed in the skeletal muscles and the gene encoding it is located on human chromosome 16p11.2.

免疫原

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial precursor recombinant protein epitope signature tag (PrEST)

应用

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

生化/生理作用

Branched chain ketoacid dehydrogenase kinase (BCKDK) plays an important role as a negative regulator of the branched-chain α-keto acid dehydrogenase complex (BCKD). It reversibly phosphorylates the subunits of BCKD and inhibits its function. This leads to a decrease in the catabolism of ketoacids of amino acids like leucine and isoleucine. Mutations in the gene encoding BCKDK are linked to neurobehavioral disorder in a form of autism.

特点和优势

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

联系

Corresponding Antigen APREST73446

外形

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

法律信息

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

WGK

WGK 1

个人防护装备

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

法规信息

常规特殊物品

分析证书(COA)

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Angels García-Cazorla et al.
Human mutation, 35(4), 470-477 (2014-01-23)
Inactivating mutations in the BCKDK gene, which codes for the kinase responsible for the negative regulation of the branched-chain α-keto acid dehydrogenase complex (BCKD), have recently been associated with a form of autism in three families. In this work, two
Eric A Muller et al.
The Journal of biological chemistry, 279(43), 44645-44655 (2004-08-11)
The committed step in the pathway for leucine, isoleucine, and valine catabolism is catalyzed by branched-chain alpha-ketoacid dehydrogenase (BCKD). This multienzyme complex is itself regulated through reversible subunit phosphorylation by a specific kinase (BCKD-kinase). Although BCKD is present in the
Michael D Neinast et al.
Cell metabolism, 29(2), 417-429 (2018-11-20)
Elevations in branched-chain amino acids (BCAAs) associate with numerous systemic diseases, including cancer, diabetes, and heart failure. However, an integrated understanding of whole-body BCAA metabolism remains lacking. Here, we employ in vivo isotopic tracing to systemically quantify BCAA oxidation in healthy

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