Howard Hughes Medical Institute, UCLA-United States Department of Energy (DOE) Institute, Departments of Biological Chemistry and Chemistry and Biochemistry, Molecular Biology Institute, UCLA, Los Angeles, California 90095.
Howard Hughes Medical Institute, Janelia Research Campus, Ashburn, Virginia 20147.
J Biol Chem. 2018 Feb 23;293(8):2888-2902. doi: 10.1074/jbc.M117.806109. Epub 2017 Dec 27.
Amyloid-β (Aβ) and human islet amyloid polypeptide (hIAPP) aggregate to form amyloid fibrils that deposit in tissues and are associated with Alzheimer's disease (AD) and type II diabetes (T2D), respectively. Individuals with T2D have an increased risk of developing AD, and conversely, AD patients have an increased risk of developing T2D. Evidence suggests that this link between AD and T2D might originate from a structural similarity between aggregates of Aβ and hIAPP. Using the cryoEM method microelectron diffraction, we determined the atomic structures of 11-residue segments from both Aβ and hIAPP, termed Aβ(24-34) WT and hIAPP(19-29) S20G, with 64% sequence similarity. We observed a high degree of structural similarity between their backbone atoms (0.96-Å root mean square deviation). Moreover, fibrils of these segments induced amyloid formation through self- and cross-seeding. Furthermore, inhibitors designed for one segment showed cross-efficacy for full-length Aβ and hIAPP and reduced cytotoxicity of both proteins, although by apparently blocking different cytotoxic mechanisms. The similarity of the atomic structures of Aβ(24-34) WT and hIAPP(19-29) S20G offers a molecular model for cross-seeding between Aβ and hIAPP.
淀粉样蛋白-β (Aβ) 和人胰岛淀粉样多肽 (hIAPP) 聚集形成淀粉样纤维,分别沉积在组织中,与阿尔茨海默病 (AD) 和 2 型糖尿病 (T2D) 相关。T2D 患者患 AD 的风险增加,反之亦然,AD 患者患 T2D 的风险增加。有证据表明,AD 和 T2D 之间的这种联系可能源于 Aβ 和 hIAPP 聚集物之间的结构相似性。使用 cryoEM 方法微电镜衍射,我们确定了具有 64%序列相似性的 Aβ 和 hIAPP 的 11 个残基片段的原子结构,分别称为 Aβ(24-34)WT 和 hIAPP(19-29)S20G。我们观察到它们的骨架原子之间具有高度的结构相似性(0.96-Å 均方根偏差)。此外,这些片段的原纤维通过自身和交叉成核诱导淀粉样形成。此外,为一个片段设计的抑制剂对全长 Aβ 和 hIAPP 显示出交叉功效,并降低了这两种蛋白质的细胞毒性,尽管显然通过阻止不同的细胞毒性机制。Aβ(24-34)WT 和 hIAPP(19-29)S20G 的原子结构相似性为 Aβ 和 hIAPP 之间的交叉成核提供了分子模型。