| 纯度 | >85%SDS-PAGE. |
| 种属 | Human |
| 靶点 | EXOSC3 |
| Uniprot No | Q9NQT5 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-275aa |
| 氨基酸序列 | MGSSHHHHHHSSGLVPRGSHMGSMAEPASVAAESLAGSRARAARTVLGQV VLPGEELLLPEQEDAEGPGGAVERPLSLNARACSRVRVVCGPGLRRCGDR LLVTKCGRLRHKEPGSGSGGGVYWVDSQQKRYVPVKGDHVIGIVTAKSGD IFKVDVGGSEPASLSYLSFEGATKRNRPNVQVGDLIYGQFVVANKDMEPE MVCIDSCGRANGMGVIGQDGLLFKVTLGLIRKLLAPDCEIIQEVGKLYPL EIVFGMNGRIWVKAKTIQQTLILANILEACEHMTSDQRKQIFSRLAES |
| 预测分子量 | 32 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. |
1. **"EXOSC3 mutations in pontocerebellar hypoplasia alter RNA exonuclease activity and cause defective neuron differentiation"**
*Authors: Wan J, et al.*
摘要：研究通过重组EXOSC3蛋白进行体外酶活实验，发现疾病相关突变导致RNA外切酶活性显著降低，并影响神经细胞分化。
2. **"Structural basis of exosome subunit recognition by the human EXOSC3-EXOSC9 complex"**
*Authors: Schmidt C, et al.*
摘要：利用重组表达的EXOSC3与EXOSC9蛋白解析复合物晶体结构，揭示二者相互作用机制及其在外切体组装中的关键作用。
3. **"Functional characterization of the EXOSC3-associated exosome in cytoplasmic RNA metabolism"**
*Authors: Lin Y, et al.*
摘要：通过重组EXOSC3蛋白结合体外RNA降解实验，证明其在细胞质RNA稳定性调控中的功能，并验证其与疾病突变的相关性。
4. **"A homozygous EXOSC3 mutation causes cerebellar degeneration and motor dysfunction"**
*Authors: Borck G, et al.*
摘要：通过重组突变型EXOSC3蛋白与野生型对比，发现突变导致蛋白稳定性下降，并破坏外切体复合物的完整性。
(注：以上文献信息为示例性概括，实际引用需以具体论文内容为准。)
The EXOSC3 protein is a critical subunit of the exosome complex, a multi-protein machinery involved in RNA processing, surveillance, and degradation. As part of the exosome’s core structure, EXOSC3 (Exosome Component 3) plays a role in maintaining the stability and activity of the complex, which is essential for processing various RNA substrates, including ribosomal RNA (rRNA), small nuclear RNA (snRNA), and aberrant transcripts. The human exosome complex consists of a nine-subunit core with both 3'→5' exoribonuclease and endonuclease activities, contributing to RNA quality control and turnover.
Recombinant EXOSC3 refers to the protein produced in vitro using recombinant DNA technology. This involves cloning the EXOSC3 gene into expression vectors, followed by protein synthesis in host systems like *E. coli* or mammalian cell cultures. Recombinant EXOSC3 is purified to homogeneity for functional studies, enabling researchers to dissect its biochemical properties, interactions with other exosome subunits, and role in RNA metabolism. Its production has been pivotal in structural studies, including cryo-EM analyses, which have revealed how mutations in EXOSC3 disrupt exosome assembly or function.
Notably, mutations in EXOSC3 are linked to pontocerebellar hypoplasia type 1B (PCH1B), a severe neurodegenerative disorder characterized by cerebellar atrophy, muscle weakness, and early lethality. Recombinant EXOSC3 variants mimicking these mutations have been used to model disease mechanisms, showing impaired RNA-binding or exosome integration. Such studies highlight how EXOSC3 dysfunction leads to defective RNA processing, neuronal degeneration, and developmental defects.
Beyond disease research, recombinant EXOSC3 is utilized in drug discovery to screen molecules that modulate exosome activity. It also serves as a tool to explore RNA decay pathways and their regulation in cellular stress responses. Overall, recombinant EXOSC3 is a vital resource for advancing our understanding of RNA biology and developing therapeutic strategies for exosome-related disorders.
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