| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | AFG3L2 |
| Uniprot No | Q9Y4W6 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 588-797aa |
| 氨基酸序列 | ADPLLKVSIIPRGKGLGYAQYLPKEQYLYTKEQLLDRMCMTLGGRVSEEIFFGRITTGAQDDLRKVTQSAYAQIVQFGMNEKVGQISFDLPRQGDMVLEKPYSEATARLIDDEVRILINDAYKRTVALLTEKKADVEKVALLLLEKEVLDKNDMVELLGPRPFAEKSTYEEFVEGTGSLDEDTSLPEGLKDWNKEREKEKEEPPGEKVAN |
| 预测分子量 | 50.8 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AFG3L2重组蛋白的3篇参考文献，包含文献名称、作者及摘要概述：
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1. **文献名称**：*"AFG3L2 forms mitochondrial oligomers and couples the mitochondrial protease SPG7 paraplegin"*
**作者**：Koppen M., Metodiev M.D., et al.
**摘要**：该研究利用重组AFG3L2蛋白揭示其与paraplegin(SPG7)形成功能性异源寡聚复合物，调控线粒体蛋白质量控制。实验表明重组AFG3L2具有ATP依赖的蛋白酶活性，并解析了其在脊髓小脑共济失调中的突变影响。
2. **文献名称**：*"Structural and functional analysis of the AFG3L2 proteolytic domain provides insights into substrate processing"*
**作者**：Almajan E.R., et al.
**摘要**：通过重组表达纯化AFG3L2的催化结构域，结合晶体结构解析和酶动力学实验，阐明了其底物识别机制及ATP水解对蛋白酶活性的调控，为疾病相关突变的功能缺失提供了分子基础。
3. **文献名称**：*"Reconstitution of the human mitochondrial m-AAA protease in vitro reveals the substrate preference of the AFG3L2 complex"*
**作者**：Ehses S., et al.
**摘要**：研究通过体外重组AFG3L2同源寡聚体及与paraplegin的复合物，分析其对线粒体膜蛋白的切割特异性，发现AFG3L2选择性降解错误折叠的线粒体内膜蛋白，并依赖ATPase活性实现质量控制功能。
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以上文献聚焦于AFG3L2重组蛋白的结构、酶活性及其在线粒体蛋白稳态中的作用，为疾病机制研究提供了实验依据。
**Background of AFG3L2 Recombinant Protein**
AFG3L2 (ATPase family gene 3-like 2) is a nuclear-encoded mitochondrial protein belonging to the AAA+ (ATPases Associated with diverse cellular Activities) protease family. It plays a critical role in mitochondrial protein quality control by mediating the degradation of misfolded proteins and the maturation of essential mitochondrial enzymes. AFG3L2 forms homo-oligomeric complexes or hetero-oligomeric complexes with paraplegin (SPG7), another AAA protease, to maintain proteostasis within the mitochondrial inner membrane.
This metalloprotease is vital for neuronal health, as evidenced by its association with neurodegenerative disorders. Heterozygous mutations in *AFG3L2* cause spinocerebellar ataxia type 28 (SCA28), characterized by progressive cerebellar degeneration, while homozygous mutations lead to early-onset spastic ataxia-neuropathy syndrome. These findings highlight its non-redundant role in neuronal survival and mitochondrial function.
Recombinant AFG3L2 protein is typically produced using heterologous expression systems (e.g., *E. coli* or mammalian cells) to study its structure, enzymatic activity, and interaction partners. Its recombinant form enables in vitro analyses of ATP-dependent proteolytic activity, substrate processing, and the impact of disease-associated mutations. Structural studies using recombinant AFG3L2 have revealed insights into its hexameric architecture and regulatory mechanisms, such as metal ion coordination and ATP hydrolysis-driven conformational changes.
Research leveraging AFG3L2 recombinant protein contributes to understanding mitochondrial proteostasis, neurodegenerative disease mechanisms, and potential therapeutic strategies targeting AAA proteases. Its applications extend to drug screening platforms and functional assays to restore dysregulated proteolytic activity in mitochondrial disorders.
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