| 纯度 | ＞90% by SDS-PAGE. |
| 种属 | Human |
| 靶点 | ACADVL |
| Uniprot No | P49748 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 41-655aa |
| 氨基酸序列 | MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF |
| 预测分子量 | 69 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. |
以下是关于ACADVL重组蛋白的3篇参考文献示例(基于真实研究方向的虚构概括，仅供参考)：
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1. **文献名称**: "Recombinant expression and functional characterization of human VLCAD in Escherichia coli"
**作者**: Smith A, et al.
**摘要**: 本研究成功在大肠杆菌中表达了带有His标签的重组人源VLCAD蛋白，通过亲和层析纯化获得高纯度蛋白。酶活性分析表明，重组蛋白可有效催化长链脂肪酸的β-氧化反应，为VLCAD缺乏症的体外酶替代疗法研究提供了基础。
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2. **文献名称**: "Structural insights into ACADVL mutations via recombinant protein crystallography"
**作者**: Johnson R, et al.
**摘要**: 通过昆虫细胞表达系统制备了重组ACADVL蛋白，并解析了其晶体结构。研究发现致病性突变(如p.Val283Ala)会导致蛋白构象改变，影响底物结合能力，揭示了VLCAD缺陷症的分子机制。
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3. **文献名称**: "Development of a yeast-based assay for screening ACADVL inhibitors using recombinant protein"
**作者**: Lee S, et al.
**摘要**: 利用酿酒酵母表达重组ACADVL蛋白，建立了一种高通量筛选模型，用于检测潜在抑制剂或激活剂。该模型成功验证了已知化合物对VLCAD酶活的调控作用，为药物开发提供了新工具。
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注：以上文献为示例性内容，实际研究中请通过PubMed或Web of Science等数据库检索真实发表的文献。
ACADVL (acyl-CoA dehydrogenase, very long chain) is a mitochondrial enzyme critical for fatty acid β-oxidation, a metabolic process that breaks down stored fats into energy. It specifically targets very-long-chain fatty acids (VLCFAs, C14-C20), catalyzing the initial step of their oxidation by removing hydrogen atoms and transferring electrons to the electron transport chain. Mutations in the ACADVL gene lead to VLCAD deficiency, an autosomal recessive disorder characterized by impaired energy production, metabolic crises, cardiomyopathy, and liver dysfunction, often manifesting in infancy or childhood.
Recombinant ACADVL protein is engineered using expression systems (e.g., E. coli, mammalian cells) to produce a purified, functional form of the enzyme for research and therapeutic applications. Its production enables detailed studies of enzyme kinetics, substrate specificity, and interactions with cofactors like FAD. Researchers use it to model VLCAD deficiency pathogenesis, screen potential drugs, or develop enzyme replacement therapies. Additionally, recombinant ACADVL serves as a reference standard in diagnostic assays to detect enzyme activity deficiencies in patient samples. Structural analyses of the recombinant protein help identify mutation-induced conformational changes, guiding personalized treatment strategies. Beyond disease-focused applications, it aids in exploring mitochondrial fatty acid metabolism regulation and metabolic flexibility in energy-demanding tissues. Advances in recombinant technology continue to enhance its stability and bioactivity, supporting its role in both basic science and translational medicine.
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