纯度 | >90%SDS-PAGE. |
种属 | Human |
靶点 | HPS4 |
Uniprot No | Q9NQG7 |
内毒素 | < 0.01EU/μg |
表达宿主 | E.coli |
表达区间 | 1-708aa |
氨基酸序列 | MATSTSTEAK SASWWNYFFL YDGSKVKEEG DPTRAGICYF YPSQTLLDQQ ELLCGQIAGV VRCVSDISDS PPTLVRLRKL KFAIKVDGDY LWVLGCAVEL PDVSCKRFLD QLVGFFNFYN GPVSLAYENC SQEELSTEWD TFIEQILKNT SDLHKIFNSL WNLDQTKVEP LLLLKAARIL QTCQRSPHIL AGCILYKGLI VSTQLPPSLT AKVLLHRTAP QEQRLPTGED APQEHGAALP PNVQIIPVFV TKEEAISLHE FPVEQMTRSL ASPAGLQDGS AQHHPKGGST SALKENATGH VESMAWTTPD PTSPDEACPD GRKENGCLSG HDLESIRPAG LHNSARGEVL GLSSSLGKEL VFLQEELDLS EIHIPEAQEV EMASGHFAFL HVPVPDGRAP YCKASLSASS SLEPTPPEDT AISSLRPPSA PEMLTQHGAQ EQLEDHPGHS SQAPIPRADP LPRRTRRPLL LPRLDPGQRG NKLPTGEQGL DEDVDGVCES HAAPGLECSS GSANCQGAGP SADGISSRLT PAESCMGLVR MNLYTHCVKG LVLSLLAEEP LLGDSAAIEE VYHSSLASLN GLEVHLKETL PRDEAASTSS TYNFTHYDRI QSLLMANLPQ VATPQDRRFL QAVSLMHSEF AQLPALYEMT VRNASTAVYA CCNPIQETYF QQLAPAARSS GFPNPQDGAF SLSGKAKQKL LKHGVNLL |
预测分子量 | 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HPS4重组蛋白的参考文献示例(注:以下内容为模拟生成,非真实文献):
1. **文献名称**:*Recombinant HPS4 Protein Expression and Interaction with HPS3 in Lysosomal Trafficking*
**作者**:Zhang Y, et al.
**摘要**:本研究通过大肠杆菌系统成功表达并纯化了重组HPS4蛋白,证实其与HPS3形成稳定的复合物(BLOC-3复合体),并证明该复合体在细胞内溶酶体相关细胞器的运输中起关键作用。
2. **文献名称**:*Functional Characterization of HPS4 in Melanosome Biogenesis Using Recombinant Protein Models*
**作者**:Wei L, et al.
**摘要**:利用杆状病毒-昆虫细胞系统表达重组HPS4蛋白,发现其缺失导致黑色素体形成异常,并通过体外实验揭示HPS4参与调控囊泡前体膜的融合过程。
3. **文献名称**:*Structural Insights into HPS4-HPS3 Complex by Cryo-EM Analysis of Recombinant Proteins*
**作者**:Smith J, et al.
**摘要**:通过冷冻电镜解析重组HPS4-HPS3复合物的三维结构,揭示了其结合界面及关键功能域,为Hermansky-Pudlak综合征的致病机制提供分子基础。
4. **文献名称**:*Recombinant HPS4 as a Therapeutic Agent for HPS4-Deficient Cell Rescue*
**作者**:Tanaka K, et al.
**摘要**:在HPS4缺陷型细胞中导入重组HPS4蛋白,成功恢复溶酶体运输功能,验证了重组蛋白在基因治疗中的潜在应用价值。
(以上文献为示例,实际研究中建议通过PubMed或Web of Science等平台检索真实文献。)
**Background of HPS4 Recombinant Protein**
The HPS4 (Hermansky-Pudlak Syndrome 4) gene encodes a protein critical for the biogenesis of lysosome-related organelles (LROs), such as melanosomes, platelet dense granules, and lysosomes. Mutations in HPS4 are linked to Hermansky-Pudlak syndrome type 4 (HPS-4), a rare autosomal recessive disorder characterized by oculocutaneous albinism, bleeding diathesis, and, in some cases, pulmonary fibrosis or immunodeficiency. The HPS4 protein forms a complex with HPS1 (BLOC-3 complex) to regulate intracellular trafficking, membrane dynamics, and cargo sorting, particularly in melanin synthesis and platelet function.
Recombinant HPS4 protein is engineered in vitro using expression systems (e.g., *E. coli*, mammalian cells) to produce purified, functional HPS4 for research. Its production enables mechanistic studies on HPS-4 pathogenesis, including how HPS4 mutations disrupt LRO formation or interorganellar communication. Researchers utilize recombinant HPS4 to analyze protein-protein interactions, assess the impact of disease-associated mutations, and explore its role in vesicular transport pathways.
Additionally, recombinant HPS4 serves as a tool for drug screening or gene therapy development targeting HPS-4. By studying its structure-function relationships, scientists aim to identify therapeutic strategies to restore normal cellular trafficking in HPS-4 patients. The protein’s study also contributes broadly to understanding lysosomal disorders and membrane trafficking biology, offering insights into related diseases like cystic fibrosis or neurodegenerative conditions. Overall, HPS4 recombinant protein is pivotal for unraveling the molecular basis of HPS-4 and advancing translational research.
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