Abstract
Neuroinflammation, typically manifest as microglial activation and astrogliosis accompanied by transcriptomic alterations, represents a common hallmark of various neurodegenerative conditions including prion diseases. Microglia play an overall neuroprotective role in prion disease, whereas reactive astrocytes with aberrant phenotypes propagate prions and contribute to prion-induced neurodegeneration. The existence of heterogeneous subpopulations and dual functions of microglia and astrocytes in prion disease make them potential targets for therapeutic intervention. A variety of neuroinflammation-related molecules are involved in prion pathogenesis. Therapeutics targeting neuroinflammation represents a novel approach to combat prion disease. Deciphering neuroinflammation in prion disease will deepen our understanding of pathogenesis of other neurodegenerative disorders.
Highlights
Prion diseases, called transmissible spongiform encephalopathies (TSE), are rare and fatal neurodegenerative disorders affecting both human and animals [1]
Systemic inflammation induced by oral infection of a by oral infection of a gastrointestinal helminth pathogen in prion-infected mice leads to gastrointestinal helminth pathogen in prion-infected mice leads to earlier appearance of inearlier appearance of interferon γ receptor 1 (IFNGR1)-expressing reactive astrocytes and terferon γ receptor 1 (IFNGR1)-expressing reactive astrocytes and accelerated onset of prion accelerated onset of prion disease [82]. These results suggest that reactive astrodisease [82]. These results suggest that reactive astrocytes in prion-infected mouse cytes in prion-infected mouse brains are more complex than the oversimplified A1 and brains are more complex than the oversimplified A1 and A2 polarization, and their molecA2 polarization, and their molecular features and functions in prion disease might differ ular features and functions in prion disease might differ from other neurodegenerative from other neurodegenerative conditions
Neuroinflammation mediated by microglia activation and astrogliosis is widely recognized as a hallmark of most types of neurodegeneration including prion diseases
Summary
Called transmissible spongiform encephalopathies (TSE), are rare and fatal neurodegenerative disorders affecting both human and animals [1]. It is well accepted that the conformational transition from PrPC to PrPSc represents a fundamental event during prion infection and propagation. The conformational differences of PrPSc determine diverse prion strains, which exhibit distinct disease phenotypes, such as clinical manifestation, incubation time, and lesion patterns, when transmitted to identical hosts. Diverse etiologies differ in the initiator of disease cascade, they share a common fundamental molecular event and similar neuropathological features, which encompass spongiform changes, neuronal loss, and neuroinflammatory responses such as astrogliosis and microglial activation as well as the accumulation and deposition of PrPSc aggregates [11]. We review the recent advances on neuroinflammation in prion disease
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