Approaches to therapy against prion diseases focused on the individual defence system

Abstract

Prion diseases are neurodegenerative disorders for which no effective treatment is available to date. Misfolded protein as aetiological agent is currently the most accepted theory. This review is focused on the hypothesis that the main basis of the progress of these disorders might fall on the individual defence system. Host protective response could convert into the early cellular event that triggers further brain tissue destruction. Specifically, neuroglial cells as main immunological cells located in brain might be influence on the pathogenic process of neurodegeneration by interaction with neurons. As consequence, neuroglia would be established as a turning point in therapeutic strategy of prion diseases. This alternative seems particularly desirable due to that they would result more effective than curative treatments trying to act on the irreversible neurodegenerating process. Moreover, all advances attained in the frame of this therapeutical approach result especially relevant for other neurodegenerative diseases such as Alzheimer’s, Parkinson’s or Huntington’s diseases, Frontotemporal dementia or Amyotrophic Lateral Sclerosis which are currently considered prion-like disorders since aberrant proteins spread throughout the brain during disease progression in all of them, and thus they may share molecular basis and mechanisms of propagation.

Keywords

• neuroglia
• prion and prion-like diseases
• therapy
• astrocytes
• microglia
• neurodegeneration
• neuroinflammation
• autoimmunity