Abstract
The presence of protein aggregates in the brain is a hallmark of neurodegenerative disorders such as Alzheimer’s disease (AD) and Parkinson’s disease (PD). Considerable evidence has revealed that the pathological protein aggregates in many neurodegenerative diseases are able to self-propagate, which may enable pathology to spread from cell-to-cell within the brain. This property is reminiscent of what occurs in prion diseases such as Creutzfeldt-Jakob disease. A widely recognized feature of prion disorders is the existence of distinct strains of prions, which are thought to represent unique protein aggregate structures. A number of recent studies have pointed to the existence of strains of protein aggregates in other, more common neurodegenerative illnesses such as AD, PD, and related disorders. In this review, we outline the pathobiology of prion strains and discuss how the concept of protein aggregate strains may help to explain the heterogeneity inherent to many human neurodegenerative disorders.
Highlights
Introduction to prions and prion strains1.1 Prion diseases and prion propagationThe transmissible spongiform encephalopathies (TSEs), referred to as prion diseases, are invariably fatal neurodegenerative disorders that affect both animals and humans
◊ Evidence is mounting that prion strains may not be structurally homogeneous, allowing strain evolution or mutation to occur when a selective pressure is present
Tau aggregates must meet a number of criteria in order to be classified as bona fide strains including existing as diverse conformers that can be stably propagated through living organisms, possessing variations in biochemical characteristics such as seeding efficiencies, toxicity, solubility, and aggregate size, and the ability to produce an array of pathologies, which would account for the range of symptoms underlying the tauopathies
Summary
1. Introduction to prions and prion strains 2. Strains of PrPSc in the human prion diseases 4. The structural biology of prion strains 5. Strains of protein aggregates in other neurodegenerative diseases
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