Twenty years of cerebellar degeneration research at the department of pathological physiology, faculty of medicine, Pilsen, Charles University.

Abstract

Mutant Lurcher mice represent an animal model of naturally occurring cerebellar degeneration. A gene mutation causes the demise of all Purkinje cells, as along with certain other types, as well as the functional elimination of the cerebellar cortex. Involvement in the research using this model of the C3H strain began at the Department of Physiology, UCL in 1995/96. It continued in scientific cooperation with other European laboratories where we obtained Lurcher mice of the B6CBA strain. The aim of the effort was first to identify the extent to which the cerebellum is involved in the higher nervous activity, i.e. cognitive and other functions. In that research, use was made of an entire array of methodological procedures to examine learning, memory, motor functions and emotional behavior. It was completed with an electrophysiological examination of the brain and special microscopic procedures. The results demonstrated that the cerebellum (aside from its traditional tasks) does in fact play a significant role in cognitive function, emotions, etc. It was further found that the neurodegenerative processes also affected the immune and endocrine functions, confirming the concept of the unity of the psycho-neuroendocrine-immune system. Surprisingly, despite their neurological impairment, the affected animals were able to learn to some extent and, make progress with physical training, improving not only their motor skills but also learning and memory, including deferring of signs of aging. These particular findings may prove useful for human medicine.