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Recombinant Human ACADSB protein

  • 中文名: 短链支链酰基辅酶A脱氢酶(ACADSB)重组蛋白
  • 别    名: ACADSB;Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
货号: PA1000-28DB
Price: ¥询价
数量:
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产品详情

纯度>90% by SDS-PAGE.
种属Human
靶点ACADSB
Uniprot NoP45954
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间34-432aa
氨基酸序列MGSSHHHHHH SSGLVPRGSH MGSHMKSSQS EALLNITNNG IHFAPLQTFT DEEMMIKSSV KKFAQEQIAP LVSTMDENSK MEKSVIQGLF QQGLMGIEVD PEYGGTGASF LSTVLVIEEL AKVDASVAVF CEIQNTLINT LIRKHGTEEQ KATYLPQLTT EKVGSFCLSE AGAGSDSFAL KTRADKEGDY YVLNGSKMWI SSAEHAGLFL VMANVDPTIG YKGITSFLVD RDTPGLHIGK PENKLGLRAS STCPLTFENV KVPEANILGQ IGHGYKYAIG SLNEGRIGIA AQMLGLAQGC FDYTIPYIKE RIQFGKRLFD FQGLQHQVAH VATQLEAARL LTYNAARLLE AGKPFIKEAS MAKYYASEIA GQTTSKCIEW MGGVGYTKDY PVEKYFRDAK IGTIYEGASN IQLNTIAKHI DAEY
预测分子量46 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

以下是与ACADSB重组蛋白相关的参考文献示例(注:部分信息为模拟内容,实际文献需通过学术数据库检索确认):

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1. **标题**: *Expression and Characterization of Recombinant Human Short/Branched Chain Acyl-CoA Dehydrogenase (ACADSB)*

**作者**: Smith J, et al.

**摘要**: 研究通过大肠杆菌系统表达并纯化人源ACADSB重组蛋白,分析其酶动力学特性,揭示其底物特异性及与短支链脂肪酸代谢的关系。

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2. **标题**: *Functional Analysis of ACADSB Mutants in 2-Methylbutyryl-CoA Dehydrogenase Deficiency*

**作者**: Tanaka K, et al.

**摘要**: 利用重组ACADSB蛋白研究致病突变对其催化活性的影响,发现特定突变导致酶活性显著降低,为相关遗传病的分子机制提供依据。

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3. **标题**: *Crystallographic Structure of ACADSB: Insights into Substrate Binding and Catalytic Mechanism*

**作者**: Zhang L, et al.

**摘要**: 通过重组ACADSB蛋白的晶体结构解析,阐明其活性中心构象及底物结合模式,为设计靶向抑制剂提供结构基础。

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4. **标题**: *Development of a High-Throughput Screening Assay for ACADSB Deficiency Using Recombinant Enzyme*

**作者**: Wang Y, et al.

**摘要**: 基于重组ACADSB蛋白建立体外高通量检测方法,用于快速筛查患者样本中的酶活性缺陷及潜在治疗药物。

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建议通过 **PubMed** 或 **Google Scholar** 检索上述关键词获取真实文献。若需具体文献协助,可提供更详细的研究方向。

背景信息

ACADSB (acyl-CoA dehydrogenase, short/branched chain) is a mitochondrial enzyme critical in fatty acid β-oxidation, specifically catalyzing the dehydrogenation of short- and branched-chain acyl-CoA substrates. It plays a vital role in metabolizing isobutyryl-CoA and 2-methylbutyryl-CoA, intermediates derived from valine and isoleucine catabolism. Mutations in the ACADSB gene are linked to 2-methylbutyryl-CoA dehydrogenase deficiency (SBCADD), a rare autosomal recessive disorder characterized by metabolic acidosis, developmental delays, and elevated metabolites in urine.

Recombinant ACADSB protein is engineered to study its structure-function relationships, enzymatic activity, and molecular mechanisms underlying SBCADD. Typically produced in bacterial (e.g., E. coli) or mammalian expression systems, the recombinant protein retains catalytic activity when purified via affinity tags (e.g., His-tag). Its production enables in vitro assays to assess substrate specificity, kinetic parameters, and inhibitory effects of potential drugs. Researchers also use it to generate antibodies for diagnostic tools or to validate genetic variants in clinical screenings.

Furthermore, recombinant ACADSB aids in elucidating interactions with electron transfer flavoprotein (ETF), essential for electron transport in mitochondria. By providing a standardized, high-purity enzyme source, it accelerates therapeutic development, including enzyme replacement strategies or small-molecule chaperones for mutation-induced misfolding. Ongoing studies focus on correlating ACADSB variants with disease severity, enhancing newborn screening protocols, and refining personalized treatment approaches for metabolic disorders.

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