| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | HADHB |
| Uniprot No | P55084 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 34-474aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MGSAAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLA RAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTP AHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMR KLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRL AAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNG IRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMG YKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHE AFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPF GATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK |
| 预测分子量 | 50 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3篇关于HADHB重组蛋白的参考文献，内容基于真实研究背景概括，但文献标题与作者为虚拟示例：
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1. **文献名称**：*"Recombinant HADHB Protein Production in E. coli for Functional Studies of Mitochondrial β-Oxidation"*
**作者**：Smith J. et al.
**摘要**：本研究利用大肠杆菌表达系统成功表达并纯化重组人源HADHB蛋白，验证其与HADHA亚基的相互作用，并证明重组蛋白具有完整的烯酰-CoA水解酶活性，为脂肪酸氧化缺陷症相关突变的功能分析提供工具。
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2. **文献名称**：*"Structural Insights into HADHB Mutants Linked to Trifunctional Protein Deficiency Using Recombinant Protein Expression"*
**作者**：Wang L. et al.
**摘要**：通过昆虫细胞表达系统制备重组HADHB突变体(p.Arg226His和p.Glu474Gln)，结合晶体学分析发现突变导致蛋白构象改变，破坏与HADHA亚基的结合能力，阐明了特定突变引发新生儿猝死的分子机制。
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3. **文献名称**：*"Optimization of HADHB Recombinant Protein Purification for High-Throughput Drug Screening"*
**作者**：Garcia R. et al.
**摘要**：开发了一种基于亲和层析和尺寸排阻色谱的两步法纯化策略，显著提高重组HADHB的产量和纯度，并应用于小分子化合物库筛选，发现两种可增强HADHB热稳定性的潜在药物候选分子。
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**备注**：以上文献为示例性质，实际文献需通过PubMed(https://pubmed.ncbi.nlm.nih.gov)或Web of Science检索关键词“HADHB recombinant”“trifunctional protein expression”获取。真实研究多集中在HADHB的酶学机制、疾病相关突变及治疗策略领域。
HADHB, also known as hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta, is a critical component of the mitochondrial trifunctional protein (TFP) complex. This complex, composed of alpha (HADHA) and beta (HADHB) subunits, catalyzes three sequential steps in long-chain fatty acid β-oxidation: long-chain 2.3-enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and long-chain 3-ketoacyl-CoA thiolase activities. HADHB specifically harbors the thiolase activity, essential for cleaving 3-ketoacyl-CoA intermediates to produce acetyl-CoA and acyl-CoA derivatives.
Recombinant HADHB protein is engineered through molecular cloning, typically expressed in bacterial (e.g., *E. coli*) or eukaryotic systems (e.g., mammalian/insect cells) to study its structure-function relationships, enzymatic mechanisms, and pathological mutations. Its production enables in vitro analysis of fatty acid oxidation disorders, particularly TFP deficiency and isolated LCHAD deficiency, which are linked to severe metabolic crises, cardiomyopathy, and sudden infant death. Researchers use recombinant HADHB to investigate mutation impacts on enzyme stability, substrate binding, and catalytic efficiency, aiding in diagnostic assay development and therapeutic exploration.
The protein’s recombinant form also supports drug screening for potential chaperone molecules to stabilize defective variants, enzyme replacement strategies, and gene therapy validation. Challenges in its production include maintaining proper folding, post-translational modifications (in eukaryotic systems), and solubility due to its mitochondrial membrane association. Current studies focus on correlating HADHB mutations with clinical severity, expanding insights into metabolic adaptations in energy-stressed tissues, and developing targeted interventions for fatty acid oxidation disorders.
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