Neuroinflammation in Prion Disease

Int J Mol Sci. 2021 Feb 23;22(4):2196. doi: 10.3390/ijms22042196.

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.

Keywords: astrogliosis; microglial activation; neurodegenerative disease; neuroinflammation; prion disease.

Publication types

  • Review

MeSH terms

  • Animals
  • Brain / metabolism
  • Brain / pathology
  • Chemokines / metabolism
  • Cytokines / metabolism
  • Gliosis / etiology
  • Humans
  • Inflammation / pathology*
  • Microglia / metabolism
  • Microglia / pathology*
  • Phagocytosis / physiology
  • Prion Diseases / metabolism
  • Prion Diseases / physiopathology*
  • Toll-Like Receptors / metabolism

Substances

  • Chemokines
  • Cytokines
  • Toll-Like Receptors