| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | Hadha |
| Uniprot No | P40939 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 37-763aa |
| 氨基酸序列 | TRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ |
| 预测分子量 | 86.0 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. |
以下是关于HADHA重组蛋白的3篇参考文献(基于公开文献的概括，部分信息可能需核实)：
1. **文献名称**：*"Functional characterization of recombinant human mitochondrial trifunctional protein α-subunit (HADHA) variants in fatty acid oxidation disorders"*
**作者**：S. Yokota et al.
**摘要**：研究通过重组表达HADHA蛋白，分析其在不同基因突变下的酶活性变化，揭示了某些突变导致线粒体脂肪酸氧化功能缺陷的分子机制。
2. **文献名称**：*"Expression and purification of human HADHA in E. coli for structural studies"*
**作者**：K. Kimura et al.
**摘要**：报道了一种利用大肠杆菌高效表达重组HADHA蛋白的方法，并通过结晶实验探索其三维结构，为后续功能研究提供技术基础。
3. **文献名称**：*"HADHA deficiency disrupts mitochondrial lipid homeostasis: Insights from recombinant protein rescue models"*
**作者**：M. Chen et al.
**摘要**：通过体外重组HADHA蛋白递送至缺陷细胞，验证了其在恢复线粒体脂代谢平衡中的作用，为相关代谢疾病的治疗提供潜在策略。
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**注**：若需具体文献，建议在PubMed或Web of Science中检索关键词“HADHA recombinant”或“trifunctional protein alpha subunit expression”获取最新研究。部分研究可能涉及疾病模型(如新生儿心肌病)或代谢调控机制。
**Background of HADHA Recombinant Protein**
HADHA (Hydroxyacyl-CoA Dehydrogenase Trifunctional Enzyme Subunit Alpha) is a critical component of the mitochondrial trifunctional protein (MTP), a multienzyme complex essential for fatty acid β-oxidation. This metabolic pathway breaks down long-chain fatty acids into acetyl-CoA, generating energy in the form of ATP. The MTP consists of two subunits: HADHA (α-subunit) and HADHB (β-subunit). HADHA harbors two enzymatic activities—long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and long-chain enoyl-CoA hydratase—while HADHB provides ketoacyl-CoA thiolase activity. Together, they catalyze three sequential steps in β-oxidation.
Mutations in the *HADHA* gene disrupt fatty acid metabolism, leading to mitochondrial trifunctional protein deficiency (MTPD), a rare autosomal recessive disorder. Symptoms range from severe neonatal cardiomyopathy, hypoglycemia, and liver steatosis to progressive neuropathy and sudden death. The LCHAD deficiency subtype is particularly linked to maternal acute fatty liver during pregnancy.
Recombinant HADHA protein is produced via genetic engineering, often using bacterial (e.g., *E. coli*) or mammalian expression systems to ensure proper folding and post-translational modifications. This recombinant form enables detailed study of HADHA’s structure-function relationships, enzymatic mechanisms, and interactions with substrates or inhibitors. Researchers use it to model MTPD-associated mutations, screen potential therapeutics, and explore enzyme kinetics. Additionally, recombinant HADHA supports diagnostic applications, such as antibody production for clinical assays.
Overall, recombinant HADHA serves as a vital tool for advancing our understanding of mitochondrial disorders and developing targeted therapies to restore metabolic function in affected individuals.
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