The role of proteolysis in Alzheimer's disease

Adv Exp Med Biol. 2000:477:379-90. doi: 10.1007/0-306-46826-3_39.

Abstract

Alzheimer's disease is characterised by the progressive deposition of the 4 kDa beta-amyloid peptide (A beta) in extracellular senile plaques in the brain. A beta is derived by proteolytic cleavage of the amyloid precursor protein (APP) by various proteinases termed secretases. alpha-Secretase is inhibited by hydroxamate-based zinc metalloproteinase inhibitors such as batimastat with I50 values in the low micromolar range, and displays many properties in common with the secretase that releases angiotensin converting enzyme. A cell impermeant biotinylated derivative of one such inhibitor completely blocked the release of APP from the surface of neuronal cells, indicating that alpha-secretase cleaves APP at the cell-surface. A range of hydroxamate-based compounds have been used to distinguish between alpha-secretase and tumour necrosis factor-alpha convertase, a member of the ADAMs (a disintegrin and metalloproteinase-like) family of zinc metalloproteinases. Recent data suggests that the presenilins may be aspartyl proteinases with the specificity of gamma-secretase. Although APP and the presenilins are present in detergent-insoluble, cholesterol- and glycosphingolipid-rich lipid rafts, they do not behave as typical lipid raft proteins, and thus it is unclear whether these membrane domains are the sites for proteolytic processing of APP.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • ADAM Proteins
  • ADAM17 Protein
  • Alzheimer Disease / drug therapy
  • Alzheimer Disease / metabolism*
  • Amyloid Precursor Protein Secretases
  • Amyloid beta-Protein Precursor / metabolism*
  • Animals
  • Aspartic Acid Endopeptidases
  • Biotinylation
  • Endopeptidases / metabolism
  • Humans
  • Hydroxamic Acids / pharmacology
  • Membrane Lipids / metabolism
  • Membrane Proteins / metabolism
  • Metalloendopeptidases / antagonists & inhibitors
  • Metalloendopeptidases / classification
  • Metalloendopeptidases / metabolism
  • Nerve Tissue Proteins / metabolism*
  • Phenylalanine / analogs & derivatives
  • Phenylalanine / pharmacology
  • Presenilin-1
  • Presenilin-2
  • Protease Inhibitors / pharmacology
  • Protease Inhibitors / therapeutic use
  • Rats
  • Thiophenes / pharmacology

Substances

  • Amyloid beta-Protein Precursor
  • BB 2116
  • Hydroxamic Acids
  • Membrane Lipids
  • Membrane Proteins
  • Nerve Tissue Proteins
  • PSEN1 protein, human
  • PSEN2 protein, human
  • Presenilin-1
  • Presenilin-2
  • Protease Inhibitors
  • Thiophenes
  • Phenylalanine
  • batimastat
  • marimastat
  • Amyloid Precursor Protein Secretases
  • Endopeptidases
  • Aspartic Acid Endopeptidases
  • BACE1 protein, human
  • ADAM Proteins
  • Metalloendopeptidases
  • ADAM17 Protein