Patterned Purkinje cell death in the cerebellum

Prog Neurobiol. 2003 Aug;70(6):473-507. doi: 10.1016/s0301-0082(03)00114-x.


The object of this review is to assemble much of the literature concerning Purkinje cell death in cerebellar pathology and to relate this to what is now known about the complex topography of the cerebellar cortex. A brief introduction to Purkinje cells, and their regionalization is provided, and then the data on Purkinje cell death in mouse models and, where appropriate, their human counterparts, have been arranged according to several broad categories--naturally-occurring and targeted mutations leading to Purkinje cell death, Purkinje cell death due to toxins, Purkinje cell death in ischemia, Purkinje cell death in infection and in inherited disorders, etc. The data reveal that cerebellar Purkinje cell death is much more topographically complex than is usually appreciated.

Publication types

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

MeSH terms

  • Animals
  • Cell Death
  • Cerebellar Cortex / pathology
  • Cerebellum / growth & development
  • Cerebellum / pathology*
  • Disease Models, Animal
  • Humans
  • Mice
  • Mutation
  • Neurotoxins / adverse effects
  • Purkinje Cells / metabolism
  • Purkinje Cells / pathology*
  • Receptors, Glutamate / metabolism


  • Neurotoxins
  • Receptors, Glutamate