Basic Science and Pathogenesis

Alzheimers Dement. 2024 Dec:20 Suppl 1:e086608. doi: 10.1002/alz.086608.

Abstract

Background: Fundamental questions remain about the key mechanisms that initiate Alzheimer's disease (AD) and the factors that promote its progression. Here we report the successful generation of the first genetically-engineered marmosets that carry knock-in (KI) point mutations in the presenilin-1 (PSEN1) gene that can be studied from birth throughout lifespan.

Method: CRISPR/Cas9 was used to generate marmosets with C410Y or A426P point mutations in PSEN1. Founders and their germline offspring are comprehensively studied longitudinally using non-invasive measures including behavior, biomarkers, neuroimaging, and multi-omics signatures.

Result: Prior to adulthood, increases in plasma Aβ were observed in PSEN1 mutation carriers relative to non-carriers. Analysis of brain revealed alterations in several enzyme-substrate interactions within the gamma secretase complex prior to adulthood.

Conclusion: Marmosets carrying KI point mutations in PSEN1 provide the opportunity to study the earliest primate-specific mechanisms that contribute to the molecular and cellular root causes of AD onset and progression.

MeSH terms

  • Alzheimer Disease* / genetics
  • Amyloid beta-Peptides / metabolism
  • Animals
  • Animals, Genetically Modified
  • Biomarkers / blood
  • Brain / metabolism
  • CRISPR-Cas Systems
  • Callithrix*
  • Disease Models, Animal
  • Female
  • Gene Knock-In Techniques
  • Humans
  • Male
  • Point Mutation
  • Presenilin-1* / genetics

Substances

  • Presenilin-1
  • Amyloid beta-Peptides
  • Biomarkers