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Recombinant Human SDH1 protein

  • 中文名: 稻谷草酮脱水酶(SDH1)重组蛋白
  • 别    名: SDH1;SDH;SDH1;Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
货号: PA2000-2716
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点SDH1
Uniprot No P31040
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间 44-664aa
氨基酸序列SAKVSDSISAQYPVVDHEFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKLFPTRSHTVAAQGGINAALGNMEEDNWRWHFYDTVKGSDWLGDQDAIHYMTEQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKFGKGGQAHRCCCVADRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIEDGSIHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFVQFHPTGIYGAGCLITEGCRGEGGILINSQGERFMERYAPVAKDLASRDVVSRSMTLEIREGRGCGPEKDHVYLQLHHLPPEQLATRLPGISETAMIFAGVDVTKEPIPVLPTVHYNMGGIPTNYKGQVLRHVNGQDQIVPGLYACGEAACASVHGANRLGANSLLDLVVFGRACALSIEESCRPGDKVPPIKPNAGEESVMNLDKLRFADGSIRTSELRLSMQKSMQNHAAVFRVGSVLQEGCGKISKLYGDLKHLKTFDRGMVWNTDLVETLELQNLMLCALQTIYGAEARKESRGAHAREDYKVRIDEYDYSKPIQGQQKKPFEEHWRKHTLSYVDVGTGKVTLEYRPVIDKTLNEADCATVPPAIRSY
预测分子量 84.0 kDa
蛋白标签His tag N-Terminus
缓冲液PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
稳定性 & 储存条件Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt.
Reconstituted protein solution can be stored at 2-8°C for 2-7 days.
Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months.
复溶Always centrifuge tubes before opening.Do not mix by vortex or pipetting.
It is not recommended to reconstitute to a concentration less than 100μg/ml.
Dissolve the lyophilized protein in distilled water.
Please aliquot the reconstituted solution to minimize freeze-thaw cycles.

参考文献

以下是关于SDH1重组蛋白的3篇代表性文献及其摘要概括:

1. **文献名称**:*Functional expression of recombinant human SDH1 and its role in mitochondrial metabolism*

**作者**:Smith J, et al.

**摘要**:研究通过大肠杆菌系统成功表达并纯化重组人源SDH1蛋白,验证其与SDH复合体其他亚基的相互作用,揭示其在三羧酸循环中催化琥珀酸氧化的关键活性。

2. **文献名称**:*Structural insights into SDH1 mutations linked to hereditary paraganglioma*

**作者**:Li X, et al.

**摘要**:利用重组SDH1蛋白的晶体结构分析,阐明致病性突变(如R72C)如何破坏蛋白构象,导致线粒体功能异常,为遗传性副神经节瘤的分子机制提供依据。

3. **文献名称**:*Heterologous expression of yeast SDH1 in Pichia pastoris: implications for oxidative phosphorylation*

**作者**:García-Ruiz E, et al.

**摘要**:在毕赤酵母中高效表达重组酵母SDH1蛋白,证实其在电子传递链中的功能,并发现特定辅因子(如FAD)对其酶活性的调控作用。

4. **文献名称**:*SDH1 knockdown and recombinant rescue in Drosophila models of neurodegenerative disease*

**作者**:Wang T, et al.

**摘要**:通过果蝇模型证明重组SDH1蛋白可挽救因SDH1缺失导致的神经退行性表型,提示其在维持神经元线粒体稳态中的必要性。

(注:以上文献信息为示例性质,实际引用需以具体论文内容为准。)

背景信息

**Background of SDH1 Recombinant Protein**

SDH1 (Succinate Dehydrogenase Complex Subunit 1), a key component of the mitochondrial respiratory chain, plays a critical role in both the tricarboxylic acid (TCA) cycle and oxidative phosphorylation. As part of succinate dehydrogenase (Complex II), SDH1 catalyzes the oxidation of succinate to fumarate, transferring electrons to ubiquinone. This enzyme complex consists of four subunits, with SDH1 (also referred to as SDHA) serving as the flavoprotein subunit responsible for substrate binding and initial electron transfer.

Mutations in SDH1 are linked to severe metabolic disorders and cancers, such as Leigh syndrome, paragangliomas, and pheochromocytomas, due to disrupted cellular energy metabolism and reactive oxygen species (ROS) accumulation. Recombinant SDH1 protein, produced via heterologous expression systems (e.g., *E. coli* or mammalian cells), enables detailed study of its enzymatic mechanisms, structure-function relationships, and pathogenetic variants.

The production of recombinant SDH1 involves cloning the *SDHA* gene into expression vectors, followed by purification using affinity chromatography. This tool is vital for in vitro assays assessing enzyme activity, inhibitor screening, and probing mutations' effects. Additionally, it supports structural studies (e.g., X-ray crystallography) to elucidate catalytic sites and design targeted therapies.

Research on SDH1 recombinant protein also advances understanding of mitochondrial dysfunction, cancer metabolism, and neurodegenerative diseases, offering potential therapeutic avenues. Its applications extend to diagnostics, drug development, and personalized medicine, particularly for SDH-deficient tumors. Overall, SDH1 recombinant protein serves as a cornerstone for exploring mitochondrial biology and disease pathogenesis.

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