| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PEX6 |
| Uniprot No | Q13608 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-980 aa |
| 活性数据 | MALAVLRVLEPFPTETPPLAVLLPPGGPWPAAELGLVLALRPAGESPAGPALLVAALEGPDAGTEEQGPGPPQLLVSRALLRLLALGSGAWVRARAVRRPPALGWALLGTSLGPGLGPRVGPLLVRRGETLPVPGPRVLETRPALQGLLGPGTRLAVTELRGRARLCPESGDSSRPPPPPVVSSFAVSGTVRRLQGVLGGTGDSLGVSRSCLRGLGLFQGEWVWVAQARESSNTSQPHLARVQVLEPRWDLSDRLGPGSGPLGEPLADGLALVPATLAFNLGCDPLEMGELRIQRYLEGSIAPEDKGSCSLLPGPPFARELHIEIVSSPHYSTNGNYDGVLYRHFQIPRVVQEGDVLCVPTIGQVEILEGSPEKLPRWREMFFKVKKTVGEAPDGPASAYLADTTHTSLYMVGSTLSPVPWLPSEESTLWSSLSPPGLEALVSELCAVLKPRLQPGGALLTGTSSVLLRGPPGCGKTTVVAAACSHLGLHLLKVPCSSLCAESSGAVETKLQAIFSRARRCRPAVLLLTAVDLLGRDRDGLGEDARVMAVLRHLLLNEDPLNSCPPLMVVATTSRAQDLPADVQTAFPHELEVPALSEGQRLSILRALTAHLPLGQEVNLAQLARRCAGFVVGDLYALLTHSSRAACTRIKNSGLAGGLTEEDEGELCAAGFPLLAEDFGQALEQLQTAHSQAVGAPKIPSVSWHDVGGLQEVKKEILETIQLPLEHPELLSLGLRRSGLLLHGPPGTGKTLLAKAVATECSLTFLSVKGPELINMYVGQSEENVREVFARARAAAPCIIFFDELDSLAPSRGRSGDSGGVMDRVVSQLLAELDGLHSTQDVFVIGATNRPDLLDPALLRPGRFDKLVFVGANEDRASQLRVLSAITRKFKLEPSVSLVNVLDCCPPQLTGADLYSLCSDAMTAALKRRVHDLEEGLEPGSSALMLTMEDLLQAAARLQPSVSEQELLRYKRIQRKFAAC |
| 分子量 | 130.5 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 0 |
| 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PEX6蛋白的3篇文献示例(基于领域知识综合概括，非真实文献)：
1. **文献名称**：**"Functional characterization of PEX6 mutations in Zellweger spectrum disorders"**
**作者**：Ebberink, M.S. et al.
**摘要**：研究分析了PEX6基因突变在过氧化物酶体生物合成障碍(如Zellweger综合征)患者中的分布及致病机制，通过体外重组表达突变型PEX6蛋白，发现ATP酶活性缺失导致其无法与PEX1形成功能性复合物，进而破坏过氧化物酶体膜蛋白转运。
2. **文献名称**：**"Recombinant human PEX6 restores peroxisomal protein import in patient-derived fibroblasts"**
**作者**：Waterham, H.R. et al.
**摘要**：通过构建重组人PEX6蛋白并导入患者成纤维细胞，验证了外源性PEX6可部分恢复过氧化物酶体基质蛋白的导入功能，证明基因治疗或蛋白替代疗法在PEX6相关疾病中的潜在应用价值。
3. **文献名称**：**"Structural insights into the AAA+ ATPase PEX1-PEX6 complex in peroxisome biogenesis"**
**作者**：Fujiki, Y. et al.
**摘要**：利用冷冻电镜技术解析了人源PEX1-PEX6复合物的三维结构，揭示了其通过ATP水解驱动过氧化物酶体膜蛋白PEX5循环的分子机制，为理解PEX6功能异常相关的疾病提供结构基础。
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**注**：以上内容为基于领域知识的概括性示例，实际文献需通过PubMed或学术数据库(如Google Scholar)以关键词“PEX6”或“human PEX6 recombinant”检索获取。
PEX6. a member of the peroxin (PEX) protein family, plays a critical role in peroxisome biogenesis and matrix protein import. As an AAA+ ATPase, it forms a heterohexameric complex with PEX1. facilitating the ATP-dependent recycling of the peroxisomal matrix protein receptor PEX5 from the peroxisomal membrane. This recycling is essential for maintaining peroxisome function, including fatty acid β-oxidation, plasmalogen synthesis, and detoxification of reactive oxygen species. Mutations in the *PEX6* gene are linked to peroxisome biogenesis disorders (PBDs), such as Zellweger syndrome spectrum (ZSS), characterized by severe neurological dysfunction, developmental delays, and multisystem abnormalities. Most pathogenic variants disrupt PEX6's ATPase activity or its interaction with PEX1. impairing peroxisomal assembly. Structurally, PEX6 contains two AAA+ domains (D1 and D2), with D2 being catalytically active. Recombinant human PEX6 protein, often expressed in *E. coli* or mammalian systems, is utilized to study molecular mechanisms of PBDs, assess mutation effects in vitro, and explore therapeutic strategies, including chaperone-based or gene-correction approaches. Its biochemical characterization continues to advance understanding of peroxisome dynamics and AAA+ protein functions in cellular homeostasis.
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