重组人SDHA蛋白(His tag) (ab226453)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE
描述
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产品名称
重组人SDHA蛋白(His tag)
参阅全部 SDHA 蛋白酶 -
纯度
> 90 % SDS-PAGE. -
表达系统
Escherichia coli -
Accession
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蛋白长度
Protein fragment -
无动物成分
No -
性质
Recombinant -
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种属
Human -
序列
SAKVSDSISAQYPVVDHEFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKL FPTRSHTVAAQGGINAALGNMEEDNWRWHFYDTVKGSDWLGDQDAIHYMT EQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKFGKGGQAHRCCCVA DRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIEDGS IHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFV QFHPTGIYGAGCLITEGCRGEGGILINSQGERFMERYAPVAKDLASRDVV SRSMTLEIREGRGCGPEKDHVYLQLHHLPPEQLATRLPGISETAMIFAGV DVTKEPIPVLPTVHYNMGGIPTNYKGQVLRHVNGQDQIVPGLYACGEAAC ASVHGANRLGANSLLDLVVFGRACALSIEESCRPGDKVPPIKPNAGEESV MNLDKLRFADGSIRTSELRLSMQKSMQNHAAVFRVGSVLQEGCGKISKLY GDLKHLKTFDRGMVWNTDLVETLELQNLMLCALQTIYGAEARKESRGAHA REDYKVRIDEYDYSKPIQGQQKKPFEEHWRKHTLSYVDVGTGKVTLEYRP VIDKTLNEADCATVPPAIRSY -
预测分子量
84 kDa including tags -
氨基酸
44 to 664 -
标签
His tag N-Terminus -
额外的序列信息
N-terminal 6xHis-SUMO tag.
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相关产品
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Compatible Secondaries
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Corresponding Antibody
技术指标
Our Abpromise guarantee covers the use of ab226453 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
SDS-PAGE
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形式
Liquid -
Concentration information loading...
制备和贮存
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稳定性和存储
Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.2
Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)
常规信息
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别名
- CMD1GG
- DHSA_HUMAN
- Flavoprotein subunit of complex II
see all -
功能
Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). -
通路
Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1. -
疾病相关
Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D) [MIM:252011]. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome.
Defects in SDHA are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
Defects in SDHA are the cause of cardiomyopathy dilated type 1GG (CMD1GG) [MIM:613642]. CMD1GG is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. -
序列相似性
Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. -
细胞定位
Mitochondrion inner membrane. - Information by UniProt
图片
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)
ab226453 尚未被引用在任何文献中。