Conformational Disease

Lancet. 1997 Jul 12;350(9071):134-8. doi: 10.1016/S0140-6736(97)02073-4.

Abstract

Several diverse disorders, including the prevalent dementias and encephalopathies, are now believed to arise from the same general disease mechanism. In each, there is abnormal unfolding and then aggregation of an underlying protein. The gradual accumulation of these aggregates and the acceleration of their formation by stress explain the characteristic late or episodic onset of the clinical disease. The understanding of these processes at the molecular level is opening prospects of more rational approaches to investigation and therapy.

Publication types

  • Review

MeSH terms

  • Amyloid / chemistry
  • Brain Diseases / etiology*
  • Brain Diseases / genetics
  • Brain Diseases / therapy
  • Dementia / etiology
  • Humans
  • Molecular Biology
  • Prions / chemistry
  • Protein Conformation*
  • Protein Folding
  • Protein Structure, Secondary
  • Proteins / chemistry
  • Proteins / genetics
  • Serpins / chemistry
  • Stress, Physiological / physiopathology
  • alpha 1-Antitrypsin / chemistry

Substances

  • Amyloid
  • Prions
  • Proteins
  • Serpins
  • alpha 1-Antitrypsin