Localization of ataxin-2 within the cerebellar cortex of the rat

Brain Res Bull. 2001 Oct-Nov;56(3-4):343-7. doi: 10.1016/s0361-9230(01)00601-3.

Abstract

Spinocerebellar ataxia type 2 is caused by a polyglutamine stretch in the protein ataxin-2 that is due to an expansion of a CAG repeat in the spinocerebellar ataxia-2 gene. The function of wild-type ataxin-2 has not been clarified. A widespread distribution of this protein throughout the brain has been reported. We examined the expression of ataxin-2 in cortical cerebellar cells of the adult rat. We performed a single label immunohistochemical study of ataxin-2 and a single label immunofluorescence study of ataxin-2 and zebrin on adjacent sections, to compare the distribution of the observed parasagittal band pattern. We also performed a double label immunofluorescence study of ataxin-2 and one of each parvalbumin, calbindin, and calretinin. Single label studies revealed that between 50% and 70% of the Purkinje cells express ataxin-2. The abundance of ataxin-2 was different between hemisphere and vermis, with a clear prevalence for the former. Furthermore, the distribution of ataxin-2-positive Purkinje cells showed a peculiar alternating parasagittal band pattern. Among the other cortical cerebellar cells only basket and granule cells showed ataxin-2 staining. Our dual label studies showed that about 50% of calbindin and more than 70% of parvalbumin-immunoreactive Purkinje cells were also labeled for ataxin-2. The uneven distribution of ataxin-2 expression in the Purkinje cell layer does not support the hypothesized link between ataxin-2 content and cell vulnerability. The differences in ataxin-2 expression among the cell types of cerebellar cortex, on the other hand, suggest a possible correlation between ataxin-2 content and cell function.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Ataxins
  • Cerebellar Cortex / chemistry*
  • Cerebellar Cortex / cytology
  • Immunohistochemistry
  • Male
  • Nerve Tissue Proteins / analysis
  • Peptides / genetics
  • Proteins / analysis*
  • Proteins / genetics
  • Purkinje Cells / chemistry
  • Rats
  • Rats, Wistar
  • Spinocerebellar Ataxias / genetics
  • Trinucleotide Repeats

Substances

  • Ataxins
  • Nerve Tissue Proteins
  • Peptides
  • Proteins
  • zebrin I
  • polyglutamine