The Departments of Neuroscience and Pharmacology, Mayo Clinic, Jacksonville, FL 32224, USA.
Departments of Chemistry and Physics, University of Illinois Chicago, Chicago, IL 60608, USA.
Molecules. 2022 Dec 12;27(24):8804. doi: 10.3390/molecules27248804.
The brains of Alzheimer's disease (AD) patients contain numerous amyloid plaques that are diagnostic of the disease. The plaques are primarily composed of the amyloidogenic peptides proteins Aβ40 and Aβ42, which are derived by the processing of the amyloid pre-cursor protein (APP) by two proteases called β-secretase and γ-secretase. Aβ42 differs from Aβ40 in having two additional hydrophobic amino acids, ILE and ALA, at the C-terminus. A small percentage of AD is autosomal dominant (ADAD) and linked either to the genes for the presenilins, which are part of γ-secretase, or APP. Because ADAD shares most pathogenic features with widespread late-onset AD, Aβ peptides have become the focus of AD research. Fibrils formed by the aggregation of these peptides are the major component of plaques and were initially targeted in AD therapy. However, the fact that the abundance of plaques does not correlate well with cognitive decline in AD patients has led investigators to examine smaller Aβ aggregates called oligomers. The low levels and heterogeneity of Aβ oligomers have made the determination of their structures difficult, but recent structure determinations of oligomers either formed or initiated in detergents have been achieved. We report here on the structures of these oligomers and suggest how they may be involved in AD.
阿尔茨海默病(AD)患者的大脑中含有许多淀粉样斑块,这些斑块是该疾病的诊断标志。这些斑块主要由淀粉样前体蛋白(APP)经两种称为β-分泌酶和γ-分泌酶的蛋白酶处理后产生的淀粉样肽蛋白 Aβ40 和 Aβ42 组成。Aβ42 在 C 末端比 Aβ40 多两个额外的疏水性氨基酸,ILE 和 ALA。一小部分 AD 是常染色体显性遗传(ADAD),与γ-分泌酶的组成部分早老素基因或 APP 基因相关。由于 ADAD 与广泛的晚发性 AD 具有大多数致病特征,因此 Aβ 肽已成为 AD 研究的焦点。这些肽聚集形成的纤维是斑块的主要成分,最初是 AD 治疗的目标。然而,斑块的丰度与 AD 患者的认知能力下降相关性不佳这一事实,促使研究人员检查称为寡聚物的较小 Aβ 聚集物。由于 Aβ 寡聚物的低水平和异质性,使得确定其结构变得困难,但最近在去污剂中形成或引发的寡聚物的结构测定已经实现。我们在此报告这些寡聚物的结构,并提出它们如何参与 AD 的机制。