Dissociation of progressive dopaminergic neuronal death and behavioral impairments by Bax deletion in a mouse model of Parkinson's diseases

PLoS One. 2011;6(10):e25346. doi: 10.1371/journal.pone.0025346. Epub 2011 Oct 17.

Abstract

Parkinson's disease (PD) is a common, late-onset movement disorder with selective degeneration of dopaminergic (DA) neurons in the substantia nigra (SN). Although the neurotoxin 6-hydroxydopamine (6-OHDA) has been used to induce progressive degeneration of DA neurons in various animal models of PD, the precise molecular pathway and the impact of anti-apoptotic treatment on this neurodegeneration are less understood. Following a striatal injection of 6-OHDA, we observed atrophy and progressive death of DA neurons in wild-type mice. These degenerating DA neurons never exhibited signs of apoptosis (i.e., caspase-3 activation and cytoplasmic release of cytochrome C), but rather show nuclear translocation of apoptosis-inducing factor (AIF), a hallmark of regulated necrosis. However, mice with genetic deletion of the proapoptotic gene Bax (Bax-KO) exhibited a complete absence of 6-OHDA-induced DA neuron death and nuclear translocation of AIF, indicating that 6-OHDA-induced DA neuronal death is mediated by Bax-dependent AIF activation. On the other hand, DA neurons that survived in Bax-KO mice exhibited marked neuronal atrophy, without significant improvement of PD-related behavioral deficits. These findings suggest that anti-apoptotic therapy may not be sufficient for PD treatment, and the prevention of Bax-independent neuronal atrophy may be an important therapeutic target.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis Inducing Factor / metabolism
  • Apoptosis*
  • Atrophy
  • Disease Models, Animal
  • Dopaminergic Neurons / pathology*
  • Mental Disorders / etiology
  • Mice
  • Mice, Knockout
  • Oxidopamine
  • Parkinson Disease / pathology
  • Parkinson Disease / physiopathology*
  • Parkinson Disease / prevention & control
  • Substantia Nigra / pathology
  • bcl-2-Associated X Protein / deficiency*
  • bcl-2-Associated X Protein / genetics

Substances

  • Apoptosis Inducing Factor
  • Bax protein, mouse
  • bcl-2-Associated X Protein
  • Oxidopamine