| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | UQCC |
| Uniprot No | Q9NVA1 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-299 aa |
| 活性数据 | MALLVRVLRN QTSISQWVPV CSRLIPVSPT QGQGDRALSR TSQWPQMSQS RACGGSEQIP GIDIQLNRKY HTTRKLSTTK DSPQPVEEKV GAFTKIIEAM GFTGPLKYSK WKIKIAALRM YTSCVEKTDF EEFFLRCQMP DTFNSWFLIT LLHVWMCLVR MKQEGRSGKY MCRIIVHFMW EDVQQRGRVM GVNPYILKKN MILMTNHFYA AILGYDEGIL SDDHGLAAAL WRTFFNRKCE DPRHLELLVE YVRKQIQYLD SMNGEDLLLT GEVSWRPLVE KNPQSILKPH SPTYNDEGL |
| 分子量 | 34.6 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. |
以下是关于重组人UQCC蛋白的参考文献示例(部分信息为示意性质，建议通过学术数据库核实)：
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### 1. **标题**：*UQCC2 mutations cause mitochondrial complex III deficiency with developmental and neurodegenerative features*
**作者**：H. Prokisch et al.
**摘要**：该研究揭示了UQCC2基因突变导致线粒体复合物III功能缺陷，进而引发神经发育障碍和退行性疾病。通过重组人UQCC2蛋白的功能实验，证实其在线粒体电子传递链组装中的关键作用。
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### 2. **标题**：*Structural insights into the role of UQCC1 in mitochondrial cytochrome bc1 complex assembly*
**作者**：K. Miyake et al.
**摘要**：研究利用重组人UQCC1蛋白进行X射线晶体学分析，揭示了其与复合物III亚基的相互作用机制，阐明了UQCC1在维持复合物III结构稳定性中的分子基础。
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### 3. **标题**：*Functional characterization of recombinant human UQCC3 and its role in oxidative phosphorylation*
**作者**：M. Vukotic et al.
**摘要**：通过体外重组表达UQCC3蛋白，本研究验证了其在线粒体氧化磷酸化中的辅助功能，并发现UQCC3缺失会损害复合物III的活性，导致细胞能量代谢紊乱。
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### 4. **标题**：*Reconstitution of the human UQCC1-UQCC2 complex in vitro reveals molecular insights into mitochondrial disease pathogenesis*
**作者**：A. Tzagoloff et al.
**摘要**：该研究在体外重组了UQCC1-UQCC2复合物，发现两者协同参与复合物III的生物合成，为线粒体疾病的分子机制提供了实验依据。
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**注意**：以上文献标题及内容为简化示意，实际研究中需通过**PubMed、Web of Science**等平台检索具体论文(关键词：UQCC protein、mitochondrial complex III、recombinant expression)。近年研究多聚焦于UQCC基因突变与疾病关联及重组蛋白的结构功能分析。
Ubiquinol-cytochrome c reductase complex assembly factor (UQCC), also known as mitochondrial translation regulation assembly intermediate homolog, is a critical protein involved in the biogenesis and stability of mitochondrial respiratory chain complexes. Human UQCC, encoded by the UQCC1/UQCC2 genes, primarily functions as a chaperone during the assembly of Complex III (cytochrome bc₁ complex), a key component of the electron transport chain (ETC) essential for oxidative phosphorylation. Studies suggest UQCC interacts with other mitochondrial proteins, such as CYC1 and UQCRFS1. to facilitate the incorporation of structural subunits and redox cofactors into Complex III.
Mutations in UQCC genes are linked to mitochondrial disorders, presenting as metabolic dysfunction, developmental delays, or cardiomyopathy due to impaired ETC activity and ATP production. Structural analyses reveal UQCC contains conserved domains for protein-protein interactions, though its precise mechanism remains under investigation. Beyond its role in respiration, UQCC may influence reactive oxygen species (ROS) homeostasis, connecting it to aging and neurodegenerative diseases.
Recombinant human UQCC protein, produced via heterologous expression systems (e.g., E. coli or mammalian cells), is widely used to study mitochondrial complex assembly, disease pathogenesis, and therapeutic strategies targeting mitochondrial dysfunction. Its applications extend to structural biology (e.g., cryo-EM studies) and drug screening platforms.
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