Wild-type and mutant alpha-synuclein induce a multi-component gene expression profile consistent with shared pathophysiology in different transgenic mouse models of PD

Exp Neurol. 2007 Mar;204(1):421-32. doi: 10.1016/j.expneurol.2006.12.005. Epub 2007 Jan 24.

Abstract

The pathophysiological processes that cause Parkinson's disease (PD) affect dopamine neurons residing in the substantia nigra with devastating consequences for normal movement. One important gene involved in both familial and sporadic PD is alpha-synuclein. We have generated three strains of alpha-synuclein transgenic mice to study the pathologic consequences of the targeted expression of mutant or wild-type human alpha-synuclein in a model system. We have analyzed gene expression patterns in these mice using high throughput microarrays in anatomical regions implicated in disease (substantia nigra and brainstem). Our study reveals gene dosage-dependent dysregulation of several genes important for the dopaminergic phenotype in mice over-expressing wild-type human alpha-synuclein in the substantia nigra at time points preceding neuronal cell death. Analysis of mutant alpha-synuclein mice at a time point when pathology is advanced reveals several new candidate genes that may play a role in neuronal demise and/or protein accumulation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Animals
  • Brain Stem / metabolism
  • Cell Death
  • Disease Models, Animal
  • Dopamine / metabolism
  • Gene Dosage
  • Gene Expression Profiling*
  • Gene Expression Regulation
  • Gene Expression*
  • Humans
  • Male
  • Mice
  • Mice, Transgenic
  • Mutation*
  • Nerve Degeneration / genetics
  • Neurons
  • Oligonucleotide Array Sequence Analysis
  • Parkinson Disease / genetics
  • Parkinson Disease / metabolism*
  • Parkinson Disease / physiopathology*
  • Reproducibility of Results
  • Substantia Nigra / metabolism
  • Substantia Nigra / physiopathology
  • alpha-Synuclein / genetics*

Substances

  • alpha-Synuclein
  • Dopamine