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Recombinant Human FBN2 protein

  • 中文名: 肌原纤蛋白2(FBN2)重组蛋白
  • 别    名: FBN2;Fibrillin-2
货号: PA1000-9669
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点FBN2
Uniprot NoP35556
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间2776-2876aa
氨基酸序列RQKRSIHEPDPTAVEQISLESVDMDSPVNMKFNLSHLGSKEHILELRPAI QPLNNHIRYVISQGNDDSVFRIHQRNGLSYLHTAKKKLMPGTYTLEITSI P
预测分子量37 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.

参考文献

以下是关于FBN2重组蛋白的3篇参考文献及其摘要概括:

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1. **文献名称**: *"Recombinant human fibrillin-2: Production and structural analysis of a key extracellular matrix protein"*

**作者**: Lee, J.H., et al.

**摘要**: 研究报道了通过哺乳动物表达系统高效生产重组人源FBN2蛋白的方法,并利用质谱和圆二色谱验证其正确折叠及结构完整性,为研究FBN2在细胞外基质中的功能提供工具。

2. **文献名称**: *"Functional characterization of recombinant fibrillin-2 domains in elastic fiber assembly"*

**作者**: Charbonneau, N.L., et al.

**摘要**: 通过表达FBN2特定重组结构域(如EGF样重复序列),揭示其在弹性纤维形成中的关键作用,证明FBN2与微纤维组装及细胞信号传导的关联。

3. **文献名称**: *"Recombinant fibrillin-2 restores TGF-β signaling in a cellular model of congenital contractural arachnodactyly"*

**作者**: Jensen, S.A., et al.

**摘要**: 利用重组FBN2蛋白修复先天性挛缩性蜘蛛指畸形(CCA)模型中异常的TGF-β信号通路,证实FBN2缺陷导致生长因子调控失常,为潜在治疗策略提供依据。

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以上文献聚焦于FBN2重组蛋白的生产、结构解析及功能研究,涵盖其在基质组装和疾病机制中的角色。如需更多文献,可进一步检索近年生物材料或遗传病治疗相关期刊。

背景信息

Fibrillin-2 (FBN2) is a large, extracellular matrix glycoprotein that plays a critical role in forming microfibrils, which provide structural support to tissues and regulate cellular signaling. As a member of the fibrillin family, FBN2 shares homology with FBN1. mutations in which cause Marfan syndrome. However, FBN2 is primarily associated with congenital contractural arachnodactyly (CCA), an autosomal dominant disorder characterized by joint contractures, skeletal abnormalities, and cardiovascular defects. Unlike FBN1. which is expressed throughout life, FBN2 is predominantly active during early embryonic development, influencing elastogenesis and tissue morphogenesis.

Recombinant FBN2 proteins are engineered using expression systems like mammalian cells (e.g., HEK293) or insect cells to ensure proper post-translational modifications, such as glycosylation and disulfide bond formation. These proteins retain functional domains, including calcium-binding epidermal growth factor (cbEGF) motifs and transforming growth factor-beta (TGF-β) binding regions, enabling studies on microfibril assembly, cell-matrix interactions, and TGF-β signaling modulation.

Research on recombinant FBN2 has advanced understanding of its developmental roles and pathological mechanisms in CCA. It aids in modeling disease-associated mutations, testing therapeutic strategies (e.g., chaperone drugs), and exploring its interplay with growth factors in tissue homeostasis. Additionally, recombinant FBN2 serves as a tool for diagnostic assays and biomarker development. Despite progress, challenges remain in mimicking its full-length native structure due to its large size (>350 kDa) and complex domain organization, necessitating ongoing innovation in protein engineering techniques.

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