BCKDK 抗体 (AA 120-151)
Our Local Distributor
北京 101111
Quick Overview for BCKDK 抗体 (AA 120-151) (ABIN391176)
抗原
See all BCKDK 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
-
-
抗原表位
- AA 120-151
-
预测反应
- M, Rat
-
纯化方法
- This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
-
免疫原
- This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK.
-
亚型
- Ig Fraction
-
-
-
-
应用备注
- WB: 1:1000. IHC-P: 1:50~100
-
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
缓冲液
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
-
储存液
- Sodium azide
-
注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
储存条件
- 4 °C,-20 °C
-
储存方法
- Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
-
有效期
- 6 months
-
-
- BCKDK (Branched Chain Ketoacid Dehydrogenase Kinase (BCKDK))
-
别名
- BCKDK
-
背景
- 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.
-
分子量
- 46360
-
基因ID
- 10295
-
NCBI登录号
- NP_001116429, NP_005872
-
UniProt
- O14874
-
途径
- SARS-CoV-2 Protein Interactome
抗原
-