Behavioural deficits in transgenic mice expressing human truncated (1-120 amino acid) alpha-synuclein

Exp Neurol. 2015 Feb:264:8-13. doi: 10.1016/j.expneurol.2014.11.003. Epub 2014 Nov 20.

Abstract

Accumulation and aggregation of alpha-synuclein in cortical and hippocampal areas is a pathological sign for dementia with Lewy bodies (DLB) and Parkinson's disease with dementia. However the mechanisms of alpha-synuclein triggered cellular dysfunction leading to the development of memory impairment is not clear. We have created a mouse model of DLB, where aggregation-prone human truncated (120 amino acid) alpha-synuclein is expressed in forebrain areas under the calcium/calmodulin-dependent protein kinase II alpha (CamKII-alpha) promoter. We have observed the presence of the transgenic protein in target forebrain areas, with small granular cytoplasmic accumulation of aggregated alpha-synuclein. This was associated with a progressive deficit in cortical-hippocampal memory tests including the Barnes maze and novel object recognition. This data suggests that low levels of aggregation prone alpha-synuclein are sufficient to induce memory deficits in mice and that forebrain regions associated with cognitive function may have an increased sensitivity to the truncated toxic form of alpha-synuclein.

Keywords: Alpha-synuclein; Dementia with Lewy bodies; Memory; Neurodegeneration; Transgenic mouse.

MeSH terms

  • Age Factors
  • Animals
  • Calcium-Calmodulin-Dependent Protein Kinase Type 2 / genetics
  • Disease Models, Animal
  • Exploratory Behavior / physiology
  • Gene Expression Regulation / genetics
  • Humans
  • Lewy Body Disease / genetics*
  • Lewy Body Disease / pathology
  • Lewy Body Disease / physiopathology*
  • Male
  • Maze Learning / physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Peptide Fragments*
  • Prosencephalon / metabolism
  • Prosencephalon / pathology
  • RNA, Messenger / metabolism
  • alpha-Synuclein / chemistry*
  • alpha-Synuclein / genetics*

Substances

  • Peptide Fragments
  • RNA, Messenger
  • alpha-Synuclein
  • Calcium-Calmodulin-Dependent Protein Kinase Type 2