MODELS OF AMYLOID SEEDING IN ALZHEIMER'S DISEASE AND SCRAPIE: Mechanistic Truths and Physiological Consequences of the Time-Dependent Solubility of Amyloid Proteins

作者: James D. Harper , Peter T. Lansbury

DOI: 10.1146/ANNUREV.BIOCHEM.66.1.385

关键词:

摘要: Ordered protein aggregation in the brain is a hallmark of Alzheimer's disease and scrapie. The disease-specific amyloid fibrils comprise primarily single protein, beta, disease, prion These proteins can be induced to form aggregates vitro that are indistinguishable from brain-derived fibrils. Consequently, much effort has been invested development model systems study details processes effects endogenous molecules have implicated disease. Selected studies this type reviewed herein. A simple mechanistic emerged for both involves nucleation-dependent polymerization. This mechanism dictates dependent on concentration time. Furthermore, formation seeded by preformed fibril. physiological consequences discussed.

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