Murine Models of Caytaxin Deficiency

Abstract

Although classically ascribed to dysfunction of the basal ganglia, work with the dystonic (dt) rat has shown that dystonia can arise from abnormal cerebellar output. The dt rat manifests generalized dystonia caused by deficiency of the neuronally restricted protein, caytaxin. Electrophysiological and biochemical studies provided evidence that defects at the climbing fiber-Purkinje cell synapse in the dt rat lead to abnormal bursting firing patterns in the cerebellar nuclei, which increase linearly with postnatal age. Mouse models of caytaxin deficiency (jittery, sidewinder, and hesitant) show variable degrees of dystonia and ataxia.

Publication Title

Movement Disorders: Genetics and Models: Second Edition

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