| 纯度 | >95%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ACVR1 |
| Uniprot No | Q04771 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 147-509aa |
| 氨基酸序列 | RKFKRRNQERLNPRDVEYGTIEGLITTNVGDSTLADLLDHSCTSGSGSGL PFLVQRTVAHQITLLECVGKGRYGEVWRGSWQGENVAVKIFSSRDEKSWF RETELYNTVMLRHENILGFIASDMTSRHSSTQLWLITHYHEMGSLYDYLQ LTTLDTVSCLRIVLSIASGLAHLHIEIFGTQGKPAIAHRDLKSKNILVKK NGQCCIADLGLAVMHSQSTNQLDVGNNPRVGTKRYMAPEVLDETIQVDCF DSYKRVDIWAFGLVLWEVARRMVSNGIVEDYKPPFYDVVPNDPSFEDMRK VVCVDQQRPNIPNRWFSDPTLTSLAKLMKECWYQNPSARLTALRIKKTLT KIDNSLDKLKTDC |
| 预测分子量 | 67 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3-4篇关于 **ACVR1重组蛋白** 的参考文献及简要摘要：
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1. **文献名称**：*Structure of the Bone Morphogenetic Protein Receptor ALK2 Reveals a Possible Structural Basis for Its Dual Role in Neural and Bone Development*
**作者**：Chaikuad A, et al.
**摘要**：该研究解析了ACVR1(ALK2)激酶结构域的晶体结构，揭示了其与配体结合的特异性机制，并探讨了ACVR1突变(如FOP相关突变)如何导致信号通路的异常激活，为理解其在骨骼和神经发育中的作用提供结构基础。
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2. **文献名称**：*ACVR1R206H receptor mutation causes fibrodysplasia ossificans progressiva by impairing BMP/SMAD signaling regulation*
**作者**：Hatsell SJ, et al.
**摘要**：文章通过重组ACVR1蛋白实验，证明FOP患者中ACVR1 R206H突变导致受体对激活素A(Activin A)的异常响应，错误激活BMP/SMAD信号通路，从而引发异位骨化，揭示了突变受体在疾病中的分子机制。
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3. **文献名称**：*Constitutively Activated ALK2 Receptor Cooperates with BMP9 in Osteogenic Differentiation*
**作者**：Hino K, et al.
**摘要**：研究利用重组ACVR1蛋白及突变体，发现其与BMP9配体的协同作用可显著增强成骨分化能力，表明ACVR1的激活状态与骨形成相关疾病的潜在治疗靶点有关。
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4. **文献名称**：*Engineered ACVR1 variants enable functional validation of receptor specificity in BMP signaling*
**作者**：Alessi Wolter D, et al.
**摘要**：通过重组表达ACVR1的多种突变体，验证了其配体结合和信号传递的特异性，为开发靶向ACVR1的抑制剂或激动剂提供了实验模型，尤其针对纤维发育不良和癌症治疗。
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以上研究覆盖了ACVR1的结构解析、突变机制、信号通路及治疗应用，均为该领域的代表性文献。
**Background of ACVR1 Recombinant Protein**
ACVR1 (Activin A Receptor Type 1), also known as ALK2. is a transmembrane serine/threonine kinase receptor primarily involved in the bone morphogenetic protein (BMP) signaling pathway. This pathway regulates critical biological processes, including embryonic development, cell differentiation, and tissue homeostasis. Structurally, the ACVR1 protein contains an extracellular ligand-binding domain, a transmembrane region, and an intracellular kinase domain. Its activation occurs upon binding to BMP ligands, leading to phosphorylation of downstream SMAD proteins and modulation of gene expression.
Recombinant ACVR1 proteins are engineered in vitro to study its function, interactions, and role in disease. These proteins are typically produced using expression systems like mammalian cells or bacteria, ensuring high purity and bioactivity. Researchers utilize recombinant ACVR1 to investigate its structural dynamics, ligand-binding specificity, and signaling mechanisms. Notably, ACVR1 has gained attention due to its association with fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder caused by gain-of-function mutations (e.g., R206H). These mutations lead to constitutive activation of the receptor, resulting in ectopic bone formation in soft tissues. Additionally, ACVR1 alterations are linked to diffuse intrinsic pontine glioma (DIPG), a pediatric brain cancer, highlighting its dual role in developmental and pathological contexts.
The development of recombinant ACVR1 has facilitated drug discovery efforts, including screening for inhibitors targeting its aberrant kinase activity. Such therapeutics aim to mitigate signaling dysregulation in FOP or cancer. Furthermore, recombinant proteins enable mechanistic studies to dissect how mutations alter receptor behavior and downstream pathways. Overall, ACVR1 recombinant tools are vital for advancing our understanding of BMP signaling and developing targeted therapies for related disorders.
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