Genetic ablation of the p66Shc adaptor protein reverses cognitive deficits and improves mitochondrial function in an APP transgenic mouse model of Alzheimer's disease

Mol Psychiatry. 2017 Apr;22(4):605-614. doi: 10.1038/mp.2016.112. Epub 2016 Jul 19.

Abstract

The mammalian ShcA adaptor protein p66Shc is a key regulator of mitochondrial reactive oxygen species (ROS) production and has previously been shown to mediate amyloid β (Aβ)-peptide-induced cytotoxicity in vitro. Moreover, p66Shc is involved in mammalian longevity and lifespan determination as revealed in the p66Shc knockout mice, which are characterized by a 30% prolonged lifespan, lower ROS levels and protection from age-related impairment of physical and cognitive performance. In this study, we hypothesized a role for p66Shc in Aβ-induced toxicity in vivo and investigated the effects of genetic p66Shc deletion in the PSAPP transgenic mice, an established Alzheimer's disease mouse model of β-amyloidosis. p66Shc-ablated PSAPP mice were characterized by an improved survival and a complete rescue of Aβ-induced cognitive deficits at the age of 15 months. Importantly, these beneficial effects on survival and cognitive performance were independent of Aβ levels and amyloid plaque deposition, but were associated with improved brain mitochondrial respiration, a reversal of mitochondrial complex I dysfunction, restored adenosine triphosphate production and reduced ROS levels. The results of this study support a role for p66Shc in Aβ-related mitochondrial dysfunction and oxidative damage in vivo, and suggest that p66Shc ablation may be a promising novel therapeutic strategy against Aβ-induced toxicity and cognitive impairment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ablation Techniques / methods
  • Alzheimer Disease / genetics
  • Alzheimer Disease / metabolism
  • Amyloid beta-Peptides / metabolism
  • Animals
  • Cognition / physiology
  • Cognition Disorders / metabolism
  • Cognitive Dysfunction / genetics*
  • Cognitive Dysfunction / metabolism
  • Disease Models, Animal
  • Mice, Knockout
  • Mice, Transgenic
  • Mitochondria / metabolism
  • Mitochondria / physiology
  • Oxidation-Reduction
  • Oxidative Stress / genetics
  • Reactive Oxygen Species / metabolism
  • Src Homology 2 Domain-Containing, Transforming Protein 1 / genetics*
  • Src Homology 2 Domain-Containing, Transforming Protein 1 / metabolism*

Substances

  • Amyloid beta-Peptides
  • Reactive Oxygen Species
  • Shc1 protein, mouse
  • Src Homology 2 Domain-Containing, Transforming Protein 1