Purkinje cell loss affects differentially the execution, acquisition and prepulse inhibition of skeletal and facial motor responses in Lurcher mice

Eur J Neurosci. 2005 Feb;21(4):979-88. doi: 10.1111/j.1460-9568.2005.03940.x.


Adult heterozygous Lurcher mice show a degeneration of almost all Purkinje cells and 90% of the granular cells of the cerebellum, resulting in ataxia or general deficits in motor coordination. These mice are therefore an excellent model for studying the role of the cerebellar cortex in motor performance, including the acquisition of new motor abilities. The performance of 3-month-old Lurcher mice was studied in various behavioural (fall, horizontal bar, rotating cylinder, and ladder), spatial orientation (water maze) and associative learning (eyelid classical conditioning) tasks and compared with that of wild-type mice. Behavioural tasks indicated a deficit for motor abilities in Lurcher mice but with some adaptation to the tests and improvement in performance. Wild-type and Lurcher mice performed swimming equally, but the latter learned the task significantly more slowly than the former. The late component of reflex blinks was smaller in amplitude and had a longer latency in Lurcher mice than in controls. Learning curves for Lurcher mice during classical conditioning of eyelid responses were similar to controls, but the amplitude of the learned response in Lurcher mice was significantly lower. The startle response to a severe tone was similar in both control and Lurcher mice but the latter were unable to produce prepulse inhibition. These results suggest that the cerebellar cortex is not indispensable for the performance of this complete set of skeletal and facial tasks, or for the acquisition of new motor abilities, but it is for the appropriate execution and adjustment of any of these motor activities.

Publication types

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

MeSH terms

  • Acoustic Stimulation / methods
  • Animals
  • Behavior, Animal / physiology
  • Cerebellar Cortex / pathology
  • Cerebellar Diseases / genetics
  • Cerebellar Diseases / physiopathology
  • Conditioning, Classical / physiology*
  • Disease Models, Animal
  • Electromyography / methods
  • Evoked Potentials / physiology
  • Evoked Potentials / radiation effects
  • Male
  • Maze Learning / physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Mutant Strains / physiology
  • Motor Activity / physiology
  • Muscle, Skeletal / physiopathology*
  • Muscle, Skeletal / radiation effects
  • Nerve Degeneration / genetics
  • Nerve Degeneration / physiopathology
  • Neural Inhibition / physiology*
  • Psychomotor Performance / physiology*
  • Purkinje Cells / physiology*
  • Reaction Time / physiology
  • Reflex, Acoustic / physiology*
  • Spatial Behavior / physiology