| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ZMPSTE24 |
| Uniprot No | O75844 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-475 aa |
| 活性数据 | MGMWASLDALWEMPAEKRIFGAVLLFSWTVYLWETFLAQRQRRIYKTTTHVPPELGQIMDSETFEKSRLYQLDKSTFSFWSGLYSETEGTLILLFGGIPYLWRLSGRFCGYAGFGPEYEITQSLVFLLLATLFSALAGLPWSLYNTFVIEEKHGFNQQTLGFFMKDAIKKFVVTQCILLPVSSLLLYIIKIGGDYFFIYAWLFTLVVSLVLVTIYADYIAPLFDKFTPLPEGKLKEEIEVMAKSIDFPLTKVYVVEGSKRSSHSNAYFYGFFKNKRIVLFDTLLEEYSVLNKDIQEDSGMEPRNEEEGNSEEIKAKVKNKKQGCKNEEVLAVLGHELGHWKLGHTVKNIIISQMNSFLCFFLFAVLIGRKELFAAFGFYDSQPTLIGLLIIFQFIFSPYNEVLSFCLTVLSRRFEFQADAFAKKLGKAKDLYSALIKLNKDNLGFPVSDWLFSMWHYSHPPLLERLQALKTMKQH |
| 分子量 | 81.2 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 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3篇关于重组人ZMPSTE24蛋白的关键文献，供参考：
---
1. **文献名称**：*Defective prelamin A processing and muscular dystrophy in Zmpste24-deficient mice*
**作者**：Pendas, A.M. et al.
**摘要**：该研究通过构建Zmpste24基因敲除小鼠模型，发现其无法有效切割prelamin A的C端，导致成熟lamin A缺乏及核形态异常。小鼠出现肌肉萎缩、运动障碍等早衰症状，提示ZMPSTE24功能缺陷与人类早衰症病理机制相关。
---
2. **文献名称**：*Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome*
**作者**：Eriksson, M. et al.
**摘要**：虽然本文主要聚焦于LMNA基因突变引发早衰症，但明确指出ZMPSTE24参与的prelamin A加工异常是疾病关键因素。未切割的farnesylated prelamin A累积导致核纤层结构紊乱，引发细胞衰老表型。
---
3. **文献名称**：*Structural basis of substrate recognition and hydrolysis by the human protease ZMPSTE24*
**作者**：Quigley, A. et al.
**摘要**：通过冷冻电镜解析ZMPSTE24三维结构，揭示其结合prelamin A的机制及催化活性位点。研究发现，ZMPSTE24独特的七次跨膜结构域构成底物识别通道，为设计靶向药物调节其活性提供结构基础。
---
这些文献涵盖了ZMPSTE24的生理功能、疾病关联及结构机制，可作为深入研究的基础。建议通过PubMed或Google Scholar检索完整文献以获取详细信息。
ZMPSTE24. a zinc metalloprotease encoded by the *ZMPSTE24* gene in humans, plays a critical role in post-translational processing of prelamin A, a precursor of nuclear scaffold protein lamin A. This enzyme catalyzes the cleavage of a farnesylated C-terminal peptide from prelamin A, enabling its maturation into functional lamin A, essential for maintaining nuclear structural integrity and chromatin organization. ZMPSTE24 also exhibits CAAX protease activity, redundantly with Rce1. in processing other farnesylated proteins.
Mutations in *ZMPSTE24* cause laminopathies such as restrictive dermopathy (RD) and Hutchinson-Gilford progeria syndrome (HGPS)-like disorders, characterized by nuclear abnormalities, premature aging, and tissue-specific dysfunctions. Mechanistically, loss of ZMPSTE24 activity leads to accumulation of unprocessed prelamin A, disrupting nuclear architecture and contributing to cellular senescence.
Structurally, ZMPSTE24 is a seven-transmembrane integral membrane protein localized to the endoplasmic reticulum. Unlike typical proteases, it uniquely combines endoprotease and exoprotease activities. Recombinant human ZMPSTE24. often expressed in mammalian or bacterial systems, is utilized to study its enzymatic mechanisms, model laminopathies, and screen potential therapeutic compounds. Its role in aging-associated pathways and nuclear dynamics makes it a target for interventions in progeroid syndromes and age-related diseases. Current research focuses on elucidating its substrate specificity, regulatory networks, and pharmacological modulation.
×
