Rodina Natalia, Hornung Simon, Sarkar Riddhiman, Suladze Saba, Peters Carsten, Schmid Philipp W N, Niu Zheng, Haslbeck Martin, Buchner Johannes, Kapurniotu Aphrodite, Reif Bernd
Helmholtz-Zentrum München (HMGU), Deutsches Forschungszentrum für Gesundheit und Umwelt, Institute of Structural Biology (STB), Ingolstädter Landstr. 1, Neuherberg 85764, Germany.
Division of Peptide Biochemistry, TUM School of Life Sciences, Technical University of Munich, Emil-Erlenmeyer-Forum 5, Freising 85354, Germany.
J Am Chem Soc. 2024 Jul 17;146(28):19077-19087. doi: 10.1021/jacs.4c03504. Epub 2024 Jul 7.
Deposition of amyloid plaques in the brains of Alzheimer's disease (AD) patients is a hallmark of the disease. AD plaques consist primarily of the beta-amyloid (Aβ) peptide but can contain other factors such as lipids, proteoglycans, and chaperones. So far, it is unclear how the cellular environment modulates fibril polymorphism and how differences in fibril structure affect cell viability. The small heat-shock protein (sHSP) alpha-B-Crystallin (αBC) is abundant in brains of AD patients, and colocalizes with Aβ amyloid plaques. Using solid-state NMR spectroscopy, we show that the Aβ40 fibril seed structure is not replicated in the presence of the sHSP. αBC prevents the generation of a compact fibril structure and leads to the formation of a new polymorph with a dynamic N-terminus. We find that the N-terminal fuzzy coat and the stability of the C-terminal residues in the Aβ40 fibril core affect the chemical and thermodynamic stability of the fibrils and influence their seeding capacity. We believe that our results yield a better understanding of how sHSP, such as αBC, that are part of the cellular environment, can affect fibril structures related to cell degeneration in amyloid diseases.
阿尔茨海默病(AD)患者大脑中淀粉样斑块的沉积是该疾病的一个标志。AD斑块主要由β-淀粉样蛋白(Aβ)肽组成,但也可包含其他因子,如脂质、蛋白聚糖和分子伴侣。到目前为止,尚不清楚细胞环境如何调节纤维多态性以及纤维结构差异如何影响细胞活力。小分子热休克蛋白(sHSP)α-B-晶状体蛋白(αBC)在AD患者大脑中含量丰富,且与Aβ淀粉样斑块共定位。通过固态核磁共振波谱,我们发现,在sHSP存在的情况下,Aβ40纤维种子结构无法复制。αBC可阻止紧密纤维结构的产生,并导致形成一种具有动态N端的新多形体。我们发现,Aβ40纤维核心中的N端模糊外壳以及C端残基的稳定性影响纤维的化学和热力学稳定性,并影响其种子形成能力。我们相信,我们的结果有助于更好地理解作为细胞环境一部分的sHSP(如αBC)如何影响与淀粉样疾病中细胞变性相关的纤维结构。