Graft-induced restoration of function in hereditary cerebellar ataxia

L. C. Triarhou, W. Zhang, W. H. Lee

Research output: Contribution to journalArticle

29 Scopus citations

Abstract

Fetal cerebellar cell suspensions, prepared from wild-type (+/+) mice, were implanted bilaterally into the cerebellum of Purkinje cell degeneration’ (pcd) mutant mice, a model of adult-onset recessively inherited cerebellar ataxia, to study the functional effects of the grafts on motor coordination and fatigue resistance in a rotating rod treadmill paradigm. The viability of transplanted Purkinje cells was verified with immunocytochemistry for calbindin-D2sk and for glutamate receptor 2/3 subunits and with in situ hybridisation histochemistry for insulin-like growth factor I mRNA, biochemical markers normally expressed by Purkinje cells in the cerebellum. Sham injections of vehicle did not appreciably modify the performance of pcd mutants in the rota-rod tests. On the other hand, bilateral cerebellar grafts led to a 3.5-fold increase in the time period that recipient pcd mice were able to stay on the rotating drum based on the comparison of mean scores (of three trials) or a 5.5-fold increase based on the comparison of maximum scores (of the three trials). These findings provide evidence for a motor enhancement in the pcd mouse model of hereditary cerebellar ataxia following intracerebellar transplantation of primordial Purkinje cells.

Original languageEnglish (US)
Pages (from-to)1827-1932
Number of pages106
JournalNeuroReport
Volume6
Issue number14
DOIs
StatePublished - Oct 1995

Keywords

  • Functional recovery
  • Hereditary cerebellar ataxia
  • Neural transplantation
  • Pcd mutant mouse

ASJC Scopus subject areas

  • Neuroscience(all)

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