重组人PDHB蛋白(ab152601)
Key features and details
- Expression system: Wheat germ
- Tags: GST tag N-Terminus
- Suitable for: WB, ELISA
描述
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产品名称
重组人PDHB蛋白 -
表达系统
Wheat germ -
蛋白长度
Protein fragment -
无动物成分
No -
性质
Recombinant -
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种属
Human -
序列
LEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQF GVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDI IFAIKKTLNI -
氨基酸
250 to 359 -
标签
GST tag N-Terminus
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技术指标
Our Abpromise guarantee covers the use of ab152601 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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应用
Western blot
ELISA
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形式
Liquid -
补充说明
This product was previously labelled as Pyruvate Dehydrogenase E1 beta subunit.
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Concentration information loading...
制备和贮存
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稳定性和存储
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.31% Glutathione, 0.79% Tris HCl
常规信息
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别名
- DKFZp564K0164
- mitochondrial
- ODPB_HUMAN
see all -
功能
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). -
疾病相关
Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy). -
细胞定位
Mitochondrion matrix. - Information by UniProt
实验方案
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
数据表及文件
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Datasheet download
文献 (0)
ab152601 尚未被引用在任何文献中。