Title:BDNF and Cerebellar Ataxia
Volume: 16
Issue: 3
Author(s): Robert Lalonde*, Magali Hernandez and Catherine Strazielle
Affiliation:
- Université de Lorraine, Laboratoire Stress, Immunité, Pathogènes EA 7300, Campus Santé, 9 avenue de la Forêt de
Haye, 54500 Vandoeuvre-les-Nancy, France
Keywords:
Neurotrophin, cerebellar development, spinocerebellar ataxia, Friedreich’s ataxia, cerebellar mutants, rotarod.
Abstract: Brain-derived neurotrophic factor (BDNF) has been proposed as a treatment for neurodegeneration,
including diseases of the cerebellum, where BDNF levels or those of its main receptor,
TrkB, are often diminished relative to controls, thereby serving as replacement therapy. Experimental
evidence indicates that BDNF signaling countered cerebellar degeneration, sensorimotor
deficits, or both, in transgenic ATXN1 mice mutated for ataxin-1, Cacna1a knock-in mice mutated
for ataxin-6, mice injected with lentivectors encoding RNA sequences against human FXN
into the cerebellar cortex, Kcnj6Wv (Weaver) mutant mice with granule cell degeneration, and rats
with olivocerebellar transaction, similar to a BDNF-overexpressing transgenic line interbred with
Cacng2stg mutant mice. In this regard, this study discusses whether BDNF is effective in cerebellar
pathologies where BDNF levels are normal and whether it is effective in cases with combined
cerebellar and basal ganglia damage.