Mechanisms of Cell Death in Cerebellar Disorders

Article type: Research Article

Authors: Wüllner, Ullrich | Evert, Bernd | Klockgether, Thomas

Affiliations: Department of Neurology, Friedrich-Wilhelms-University Bonn, Sigmund-Freud-Strasse 25, D-53105, Bonn, Germany

Abstract: Recent advances in molecular genetic and cellular biology have provided new insights into the mechanisms leading to neuronal dysfunction and cell death in the disorders of the cerebellum. Trinucleotide repeat expansions have been identified as an important cause of inherited cerebellar ataxias and mechanisms involved in apoptotic cell death have become centerstage of scientific interest. In the present article, we review the recent findings in trinucleotide repeat disorders and address the possible link of glutamate neurotoxicity, impaired Ca2+ homeostasis and apoptosis.

Keywords: spinocerebellar ataxia, trinucleotide repeats, calcium, apoptosis, glutamate

Journal: Restorative Neurology and Neuroscience, vol. 13, no. 1-2, pp. 69-73, 1998