Early pathogenic event of Alzheimer's disease documented in iPSCs from patients with PSEN1 mutations

Oncotarget. 2017 Jan 31;8(5):7900-7913. doi: 10.18632/oncotarget.13776.

Abstract

Alzheimer's disease (AD) is the most common age-related dementia characterized by progressive neuronal loss. However, the molecular mechanisms for the neuronal loss is still debated. Here, we used induced pluripotent stem cells (iPSCs) derived from somatic cells of familial AD patients carrying PSEN1 mutations to study the early pathogenic event of AD. We found that premature neuronal differentiation with decreased proliferation and increased apoptosis occured in AD-iPSC-derived neural progenitor cells (AD-NPCs) once neuronal differentiation was initiated, together with higher levels of Aβ42 and phosphorylated tau. Premature neuronal differentiation in AD-NPCs was caused by PSEN1 mutations and might be correlated to multiple dysregulated processes including but not limited to Wnt-Notch pathway. Our study documented previously unappreciated early NPC dysfunction in AD-NPCs, providing valuable new insights into the early mechanisms underlying AD pathogenesis.

Keywords: Alzheimer’s disease; apoptosis; induced pluripotent stem cells; neural progenitor cells; premature neuronal differentiation.

MeSH terms

  • Alzheimer Disease / genetics*
  • Alzheimer Disease / metabolism
  • Alzheimer Disease / pathology*
  • Amyloid beta-Peptides / metabolism
  • Apoptosis
  • Cell Line
  • Cell Proliferation
  • Female
  • Genetic Markers
  • Genetic Predisposition to Disease
  • Humans
  • Induced Pluripotent Stem Cells / metabolism
  • Induced Pluripotent Stem Cells / pathology*
  • Middle Aged
  • Mutation*
  • Neural Stem Cells / metabolism
  • Neural Stem Cells / pathology*
  • Neurogenesis
  • Peptide Fragments / metabolism
  • Phenotype
  • Phosphorylation
  • Presenilin-1 / genetics*
  • Time Factors
  • tau Proteins / metabolism

Substances

  • Amyloid beta-Peptides
  • Genetic Markers
  • MAPT protein, human
  • PSEN1 protein, human
  • Peptide Fragments
  • Presenilin-1
  • amyloid beta-protein (1-42)
  • tau Proteins