Overexpression of a Hu-bcl-2 transgene in Lurcher mutant mice delays Purkinje cell death

C R Acad Sci III. 1998 Aug;321(8):633-40. doi: 10.1016/s0764-4469(98)80002-4.

Abstract

Cerebellar Purkinje cells in the heterozygous Lurcher mutant undergo cell autonomous degeneration beginning in the second week of postnatal development and becoming almost total around 30-45 days. The Lurcher mutation was recently identified as gain-of-function defect in the delta 2 glutamate receptor causing a constitutive current leak, suggesting that +/Lc Purkinje cells die by an excitotoxic mechanism. In previous studies we have shown that overexpression of bcl-2, a key regulator of cell death, in the heterozygous Lurcher mutant does not prevent +/Lc Purkinje cell death. To investigate further the mechanisms of +/Lc Purkinje cell death, we have crossed +/Lc mutants with a second line of Hu-bcl-2 transgenics (NSE73a) that shows an earlier onset of transgene expression and higher expression levels. Analysis of eight +/Lc-NSE73a mutants (4 at 2 months and 4 at 5-6 months) showed that Hu-bcl-2 overexpression delayed, but ultimately could not prevent +/Lc Purkinje cell death.

Publication types

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

MeSH terms

  • Animals
  • Cell Death / physiology*
  • Female
  • Gene Expression Regulation, Developmental / physiology*
  • Genes, bcl-2*
  • Male
  • Mice
  • Mice, Neurologic Mutants
  • Nerve Degeneration / physiopathology
  • Purkinje Cells / metabolism*
  • Purkinje Cells / pathology