The cerebral proteopathies: neurodegenerative disorders of protein conformation and assembly

Mol Neurobiol. 2000 Feb-Apr;21(1-2):83-95. doi: 10.1385/MN:21:1-2:083.

Abstract

The abnormal assembly and deposition of specific proteins in the brain is the probable cause of most neurodegenerative disease afflicting the elderly. These "cerebral proteopathies" include Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), prion diseases, and a variety of other disorders. Evidence is accumulating that the anomalous aggregation of the proteins, and not a loss of protein function, is central to the pathogenesis of these diseases. Thus, therapeutic strategies that reduce the production, accumulation, or polymerization of pathogenic proteins might be applicable to a wide range of some of the most devastating diseases of old age.

Publication types

  • Review

MeSH terms

  • Amyloid beta-Peptides / chemistry
  • Amyloid beta-Peptides / metabolism*
  • Amyloidosis / genetics
  • Amyloidosis / pathology
  • Amyloidosis / therapy
  • Animals
  • Brain / metabolism*
  • Brain / pathology*
  • Humans
  • Prion Diseases / genetics
  • Prion Diseases / metabolism*
  • Prion Diseases / pathology*
  • Prion Diseases / therapy
  • Protein Conformation

Substances

  • Amyloid beta-Peptides