Bcl-2 expression using retrograde transport of adenoviral vectors inhibits cytochrome c-release and caspase-1 activation in motor neurons of mutant superoxide dismutase 1 (G93A) transgenic mice

Neurosci Lett. 2003 Oct 16;350(1):17-20. doi: 10.1016/s0304-3940(03)00817-6.

Abstract

We explored a possible mechanism of the neuro-protective effects of exogenous human Bcl-2 expression on motor neurons of transgenic mice expressing human Cu/Zn superoxide dismutase with a G93A mutation (G93A mice), using retrograde transport and a Cre-loxP recombination system employing adenoviral vectors. We examined the cellular localization of cytochrome c and caspase-1 using immunohistochemical study, in motor neurons of hypoglossal nuclei of G93A mice at 15 weeks after inoculation with the adenoviral vectors, at which time over-expressed exogenous Bcl-2 declined to reach the baseline of intrinsic Bcl-2. We found that a significant number of neurons showed more faint and punctate immunostaining against cytochrome c and significantly less neurons showed immunoreactivity against activated caspase-1, compared with those of mice without inoculation. These results suggest that transient exogenous Bcl-2 expression at the early stage of the disease protects against motor neuronal degeneration in G93A mice by retarding translocation of cytochrome c into the cytosol, and regulating caspase-1 for a substantial period.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenoviridae / genetics
  • Alanine / genetics
  • Animals
  • Axonal Transport
  • Brain Stem / metabolism
  • Brain Stem / virology
  • Caspase 1 / metabolism*
  • Cell Count
  • Cytochrome c Group / metabolism*
  • Enzyme Activation / drug effects
  • Gene Transfer Techniques
  • Genetic Vectors / genetics
  • Glycine / genetics
  • Humans
  • Immunohistochemistry
  • Integrases
  • Mice
  • Mice, Neurologic Mutants
  • Mice, Transgenic
  • Motor Neurons / drug effects*
  • Motor Neurons / enzymology
  • Motor Neurons / metabolism
  • Motor Neurons / virology
  • Mutation
  • Proto-Oncogene Proteins c-bcl-2 / biosynthesis
  • Proto-Oncogene Proteins c-bcl-2 / genetics
  • Proto-Oncogene Proteins c-bcl-2 / pharmacology*
  • Superoxide Dismutase / genetics*
  • Superoxide Dismutase-1
  • Viral Proteins

Substances

  • Cytochrome c Group
  • Proto-Oncogene Proteins c-bcl-2
  • SOD1 protein, human
  • Viral Proteins
  • Sod1 protein, mouse
  • Superoxide Dismutase
  • Superoxide Dismutase-1
  • Cre recombinase
  • Integrases
  • Caspase 1
  • Alanine
  • Glycine