Downstream mechanisms triggered by mitochondrial dysfunction in the basal ganglia: From experimental models to neurodegenerative diseases

Paolo Gubellini, Barbara Picconi, Massimiliano Di Filippo, Paolo Calabresi

Research output: Contribution to journalArticle

Abstract

Mitochondrial dysfunctions have been implicated in the cellular processes underlying several neurodegenerative disorders affecting the basal ganglia. These include Huntington's chorea and Parkinson's disease, two highly debilitating motor disorders for which recent research has also involved gene mutation linked to mitochondrial deficits. Experimental models of basal ganglia diseases have been developed by using toxins able to disrupt mitochondrial function: these molecules act by selectively inhibiting mitochondrial respiratory complexes, uncoupling cellular respiration. This in turn leads to oxidative stress and energy deficit that trigger critical downstream mechanisms, ultimately resulting in neuronal vulnerability and loss. Here we review the molecular and cellular downstream effects triggered by mitochondrial dysfunction, and the different experimental models that are obtained by the administration of selective mitochondrial toxins or by the expression of mutant genes.

Original languageEnglish
Pages (from-to)151-161
Number of pages11
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1802
Issue number1
DOIs
Publication statusPublished - Jan 2010

Keywords

  • 3-nitropropionic acid
  • Dopamine
  • Huntingtin
  • Huntington's chorea
  • Long-term potentiation
  • Parkin
  • Parkinson's disease
  • PINK1
  • Rotenone
  • Striatum

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine

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