重组人PDHA1蛋白(ab125602)
Key features and details
- Expression system: Escherichia coli
- Purity: > 85% Densitometry
- Tags: His tag N-Terminus
- Suitable for: Functional Studies, WB, SDS-PAGE
描述
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产品名称
重组人PDHA1蛋白 -
纯度
> 85 % Densitometry. -
表达系统
Escherichia coli -
Accession
-
蛋白长度
Full length protein -
无动物成分
No -
性质
Recombinant -
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种属
Human -
预测分子量
47 kDa -
氨基酸
30 to 390 -
标签
His tag N-Terminus
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相关产品
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Recombinant Protein
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Related Products
技术指标
Our Abpromise guarantee covers the use of ab125602 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
Functional Studies
Western blot
SDS-PAGE
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形式
Liquid -
补充说明
ab125602 (Human Pyruvate Dehydrogenase E1-alpha subunit full length protein) can be utilized as a substrate for the following active protein Kinases:
ab125560 (Active human PDK4 full length protein)
ab125580 (Active human Mitochondrial Pyruvate dehydrogenase kinase 1 full length protein)
ab125592 (Active human PDK2 full length protein)
ab125606 (Active human PDK3 full length protein) -
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: 7.00
Preservative: 1.02% Imidazole
Constituents: 0.002% PMSF, 0.81% Sodium phosphate, 0.004% DTT, 25% Glycerol (glycerin, glycerine), 1.75% Sodium chloride
常规信息
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别名
- ODPA_HUMAN
- PDH
- PDHA
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). -
组织特异性
Ubiquitous. -
疾病相关
Defects in PDHA1 are a cause of pyruvate decarboxylase 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 (X-LS).
Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes. -
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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SDS download
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Datasheet download
文献 (0)
ab125602 尚未被引用在任何文献中。