| 纯度 | ＞90% by SDS-PAGE. |
| 种属 | Human |
| 靶点 | ACAD8 |
| Uniprot No | Q9UKU7 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 23-415aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MGSLVQTGHR SLTSCIDPSM GLNEEQKEFQ KVAFDFAARE MAPNMAEWDQ KELFPVDVMR KAAQLGFGGV YIQTDVGGSG LSRLDTSVIF EALATGCTST TAYISIHNMC AWMIDSFGNE EQRHKFCPPL CTMEKFASYC LTEPGSGSDA ASLLTSAKKQ GDHYILNGSK AFISGAGESD IYVVMCRTGG PGPKGISCIV VEKGTPGLSF GKKEKKVGWN SQPTRAVIFE DCAVPVANRI GSEGQGFLIA VRGLNGGRIN IASCSLGAAH ASVILTRDHL NVRKQFGEPL ASNQYLQFTL ADMATRLVAA RLMVRNAAVA LQEERKDAVA LCSMAKLFAT DECFAICNQA LQMHGGYGYL KDYAVQQYVR DSRVHQILEG SNEVMRILIS RSLLQE |
| 预测分子量 | 45 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ACAD8重组蛋白的3篇参考文献及其摘要概括：
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1. **文献名称**：*"Cloning and expression of human isobutyryl-CoA dehydrogenase and its involvement in valine metabolism"*
**作者**：He M. 等 (2002)
**摘要**：该研究首次报道了人源ACAD8基因的克隆及重组蛋白在大肠杆菌中的表达，证实其催化异丁酰辅酶A的脱氢反应，并揭示了ACAD8在缬氨酸代谢中的关键作用。
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2. **文献名称**：*"Functional characterization of mutations in ACAD8 causing isobutyryl-CoA dehydrogenase deficiency"*
**作者**：Nguyen T.V. 等 (2011)
**摘要**：通过重组表达突变型ACAD8蛋白，分析了丙酸尿症患者中ACAD8基因突变的酶活性变化，发现部分突变导致蛋白稳定性及底物结合能力显著下降。
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3. **文献名称**：*"Structural insights into substrate recognition by human isobutyryl-CoA dehydrogenase (ACAD8)"*
**作者**：Sampathkumar S. 等 (2013)
**摘要**：利用重组ACAD8蛋白进行X射线晶体学研究，解析了其三维结构，阐明了底物特异性结合口袋的分子机制，为代谢疾病的药物设计提供结构基础。
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ACAD8 (Acyl-CoA Dehydrogenase Family Member 8) is a mitochondrial enzyme belonging to the acyl-CoA dehydrogenase family, which plays a critical role in amino acid and fatty acid metabolism. Specifically, ACAD8 catalyzes the dehydrogenation of 2-methylbutyryl-CoA to tiglyl-CoA in the isoleucine catabolic pathway, a key step in the breakdown of branched-chain amino acids. This reaction is essential for generating energy via the mitochondrial electron transport chain. Dysregulation of ACAD8 activity has been linked to metabolic disorders, particularly 2-methylbutyryl-CoA dehydrogenase deficiency (OMIM 610006), an autosomal recessive condition characterized by elevated levels of 2-methylbutyrylglycine in urine, metabolic acidosis, and developmental delays.
Recombinant ACAD8 protein is commonly produced in heterologous expression systems (e.g., E. coli or mammalian cells) for functional studies and therapeutic development. Its recombinant form retains enzymatic activity and structural features of the native protein, including conserved FAD-binding domains and catalytic residues. Researchers utilize this protein to investigate substrate specificity, mutation effects, and potential pharmacological chaperones for ACAD8-related disorders. Furthermore, recombinant ACAD8 serves as a valuable tool for developing diagnostic assays and screening metabolic modulators. Recent studies also explore its potential in gene therapy approaches to restore enzymatic function in deficient models. Structural analyses via X-ray crystallography and cryo-EM using recombinant ACAD8 have advanced understanding of its mechanism and evolutionary relationships within the acyl-CoA dehydrogenase superfamily.
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