Generation of C-terminally truncated amyloid-beta peptides is dependent on gamma-secretase activity

J Neurochem. 2002 Aug;82(3):563-75. doi: 10.1046/j.1471-4159.2002.00985.x.

Abstract

Aberrant production of amyloid-beta peptides by processing of the beta-amyloid precursor protein leads to the formation of characteristic extracellular protein deposits which are thought to be the cause of Alzheimer's disease. Therefore, inhibiting the key enzymes responsible for amyloid-beta peptide generation, beta- and gamma-secretase may offer an opportunity to intervene with the progression of the disease. In human brain and cell culture systems a heterogeneous population of amyloid-beta peptides with various truncations is detected and at present, it is unclear how they are produced. We have used a combination of surface enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF MS) and a specific inhibitor of gamma-secretase to investigate whether the production of all amyloid-beta peptide species requires the action of gamma-secretase. Using this approach, we demonstrate that the production of all truncated amyloid-beta peptides except those released by the action of the nonamyloidogenic alpha-secretase enzyme or potentially beta-site betaAPP cleaving enzyme 2 depends on gamma-secretase activity. This indicates that none of these peptides are generated by a separate enzyme entity and a specific inhibitor of the gamma-secretase enzyme should havethe potential to block the generation of all amyloidogenicpeptides. Furthermore in the presence of gamma-secretase inhibitors, the observation of increased cleavage of the membrane-bound betaAPP C-terminal fragment C99 by alpha-secretase suggests that during its trafficking C99 encounters compartments in which alpha-secretase activity resides.

MeSH terms

  • Alzheimer Disease / metabolism*
  • Amino Acid Sequence
  • Amyloid Precursor Protein Secretases
  • Amyloid beta-Peptides / chemistry
  • Amyloid beta-Peptides / metabolism*
  • Amyloid beta-Protein Precursor / genetics
  • Amyloid beta-Protein Precursor / metabolism
  • Antibodies, Monoclonal / chemistry
  • Antibody Specificity
  • Aspartic Acid Endopeptidases
  • Cell Line
  • Culture Media, Conditioned / chemistry
  • Culture Media, Conditioned / metabolism
  • Endopeptidases / drug effects*
  • Endopeptidases / metabolism
  • Enzyme Activation / drug effects
  • Enzyme Inhibitors / chemistry
  • Enzyme Inhibitors / pharmacology*
  • Fluorobenzenes / pharmacology
  • Humans
  • Kidney / cytology
  • Kidney / metabolism
  • Mass Spectrometry
  • Metalloendopeptidases / antagonists & inhibitors
  • Molecular Sequence Data
  • Neuroblastoma / metabolism
  • Pentanoic Acids / pharmacology
  • Peptide Fragments / analysis
  • Peptide Fragments / biosynthesis*
  • Peptide Fragments / chemistry
  • Peptide Fragments / genetics
  • Peptide Fragments / metabolism
  • Peptides*
  • Phenylalanine / analogs & derivatives*
  • Phenylalanine / pharmacology
  • Recombinant Proteins*
  • Thiophenes / pharmacology
  • Transfection

Substances

  • 1,2(S)-(2(S)-(2-(3,5-difluorophenyl)acetylamino)propionylamino)-4-methylpentanoic acid methyl ester
  • Amyloid beta-Peptides
  • Amyloid beta-Protein Precursor
  • Antibodies, Monoclonal
  • Culture Media, Conditioned
  • Enzyme Inhibitors
  • Fluorobenzenes
  • MW167
  • Pentanoic Acids
  • Peptide Fragments
  • Peptides
  • Recombinant Proteins
  • SPA4CT protein, recombinant
  • Thiophenes
  • Phenylalanine
  • batimastat
  • Amyloid Precursor Protein Secretases
  • Endopeptidases
  • Aspartic Acid Endopeptidases
  • BACE1 protein, human
  • Metalloendopeptidases