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Characteristics of the nervous system of mice with a Lurcher mutation
Boubín, Josef ; Bendová, Zdeňka (advisor) ; Tůma, Jan (referee)
The glutamate receptor δ 2 (GluRδ2) is expressed in dendrites of Purkinje cells localized in the cerebellar cortex. Correct function of GluRδ2 is necessary for cerebellar development, synapse formation between parallel fibers of the granular and Purkinje cells and for inducing long term depression important in memory formation. Lurcher mutation, localized to 6th autosomal chromosome, transforms GluRδ2 into constitutively open ion channel by amino acid substitution in third transmembrane domain. As a result, almost complete disappearance of Purkinje cells population and a large degeneration of granular cells and olivary neurons occurs. Mice impaired by Lurcher mutation have lower body weight and reduced litter size. Fertility of males is not affected. Lurcher mutants display extensive behavioral deficits. Mice suffer from ataxia typical for cerebellar neurodegenerations. They have reduced physical performance, impaired spatial orientation and learning capabilities. The aim of this work is to summarize recent knowledge about Lurcher mutation from molecular basis to behavioral manifestation. Specific characteristics of this degeneration allow us to investigate influences of neurodegenerative cerebellar disorders on cognitive functions of the brain as a whole, study causing factors and treatment...

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