Order NO. D163672
产品描述
概述
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.
应用
酶联免疫吸附测定(ELISA),免疫印迹(WB),免疫组织化学(IHC)
属性
保存缓冲液 |
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
|
运输条件 |
2-8°C
|
靶点 Uniprot 登记号 |
P11182
|
靶点基因 ID |
1629
|
靶点全称 |
dihydrolipoamide branched chain transacylase E2
|
靶点别名 |
E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2
|
靶点研究领域 |
Metabolism
|
亚型 |
Immunogen-specific rabbit IgG
|