Amyloid precursor protein transgenic mouse models and Alzheimer's disease: understanding the paradigms, limitations, and contributions

Alzheimers Dement. 2009 Jul;5(4):340-7. doi: 10.1016/j.jalz.2009.03.002.

Abstract

Transgenic (Tg) mice that overexpress mutant familial Alzheimer's disease (AD) amyloid precursor protein (APP) genes have contributed to an understanding of dementia pathology, and support the amyloid cascade hypothesis. Although many sophisticated mice APP models exist, none recapitulates AD cellular and behavioral pathology. The morphological resemblance to AD amyloidosis is impressive, but fundamental biophysical and biochemical properties of the APP/Abeta produced in Tg mice differ substantially from those of humans. The greater resilience of Tg mice in the presence of substantial Abeta burdens suggests that levels and forms deleterious to human neurons are not as noxious in these models. Transgenic mice were widely used for testing AD therapeutic agents, and demonstrated promising results. Unfortunately, clinical trials resulted in unforeseen adverse events or negative therapeutic outcomes. The disparity between success and failure is in part attributable to evolutionary divergence between humans and rodents. These observations suggest that the pathogenesis of AD is by far more intricate than can be explained by a straightforward accumulation of Abeta.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Alzheimer Disease / drug therapy
  • Alzheimer Disease / genetics*
  • Alzheimer Disease / metabolism*
  • Amyloid beta-Peptides / genetics
  • Amyloid beta-Peptides / metabolism
  • Amyloid beta-Protein Precursor / genetics*
  • Amyloid beta-Protein Precursor / metabolism*
  • Animals
  • Brain / metabolism
  • Brain / pathology
  • Brain / physiopathology
  • Clinical Trials as Topic / adverse effects
  • Clinical Trials as Topic / methods
  • Disease Models, Animal*
  • Drug Evaluation, Preclinical / methods
  • Humans
  • Mice
  • Mice, Transgenic*
  • Neurons / metabolism
  • Neurons / pathology
  • Species Specificity
  • Treatment Failure

Substances

  • Amyloid beta-Peptides
  • Amyloid beta-Protein Precursor