Cell-to-cell Transmission of Pathogenic Proteins in Neurodegenerative Diseases

Nat Med. 2014 Feb;20(2):130-8. doi: 10.1038/nm.3457.

Abstract

A common feature of many neurodegenerative diseases is the deposition of β-sheet-rich amyloid aggregates formed by proteins specific to these diseases. These protein aggregates are thought to cause neuronal dysfunction, directly or indirectly. Recent studies have strongly implicated cell-to-cell transmission of misfolded proteins as a common mechanism for the onset and progression of various neurodegenerative disorders. Emerging evidence also suggests the presence of conformationally diverse 'strains' of each type of disease protein, which may be another shared feature of amyloid aggregates, accounting for the tremendous heterogeneity within each type of neurodegenerative disease. Although there are many more questions to be answered, these studies have opened up new avenues for therapeutic interventions in neurodegenerative disorders.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amyloidogenic Proteins / classification
  • Amyloidogenic Proteins / metabolism*
  • Cell Communication / physiology*
  • Humans
  • Models, Biological*
  • Neurodegenerative Diseases / physiopathology*
  • Protein Transport / physiology
  • Proteostasis Deficiencies / physiopathology*

Substances

  • Amyloidogenic Proteins