Elucidation of molecular mechanisms by which amyloid β(1)(-)(42) fibrils exert cell toxicity

阐明淀粉样β(1)(-)(42)原纤维发挥细胞毒性的分子机制

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Abstract

Abrupt aggregation of amyloid β(1)(-)(42) (Aβ(1)(-)(42)) peptide in the frontal lobe is the expected underlying cause of Alzheimer's disease (AD). β-Sheet-rich oligomers and fibrils formed by Aβ(1)(-)(42) exert high cell toxicity. A growing body of evidence indicates that lipids can uniquely alter the secondary structure and toxicity of Aβ(1)(-)(42) aggregates. At the same time, underlying molecular mechanisms that determine this difference in toxicity of amyloid aggregates remain unclear. Using a set of molecular and biophysical assays to determine the molecular mechanism by which Aβ(1)(-)(42) aggregates formed in the presence of cholesterol, cardiolipin, and phosphatidylcholine exert cell toxicity. Our findings demonstrate that rat neuronal cells exposed to Aβ(1)(-)(42) fibrils formed in the presence of lipids with different chemical structure exert drastically different magnitude and dynamic of unfolded protein response (UPR) in the endoplasmic reticulum (ER) and mitochondria (MT). We found that the opposite dynamics of UPR in MT and ER in the cells exposed to Aβ(1)(-)(42): cardiolipin fibrils and Aβ(1)(-)(42) aggregates formed in a lipid-free environment. We also found that Aβ(1)(-)(42): phosphatidylcholine fibrils upregulated ER UPR simultaneously downregulating the UPR response of MT, whereas Aβ(1)(-)(42): cholesterol fibrils suppressed the UPR response of ER and upregulated UPR response of MT. We also observed progressively increasing ROS production that damages mitochondrial membranes and other cell organelles, ultimately leading to cell death.

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