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Purkinje cell expression of a mutant SCA1 allele in transgenic mice leads to disparate effects on motor behaviours followed by a progressive cerebellar dysfunction and histological abnormalities

Clark, HB; Burright, EN; Yunis, WS; Larson, S; Wilcox, C; Hartman, B; Matilla, A; ... Orr, HT; + view all (1997) Purkinje cell expression of a mutant SCA1 allele in transgenic mice leads to disparate effects on motor behaviours followed by a progressive cerebellar dysfunction and histological abnormalities. Journal of Neuroscience , 17 (19) 7385 - 7395. Green open access

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Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurological disorder caused by the expansion of a CAG repeat encoding a polyglutamine tract. Work presented here describes the behavioral and neuropathological course seen in mutant SCA1 transgenic mice. Behavioral tests indicate that at 5 weeks of age mutant mice have an impaired performance on the rotating rod in the absence of deficits in balance and coordination. In contrast, these mutant SCA1 mice have an increased initial exploratory behavior. Thus, expression of the mutant SCA1 allele within cerebellar Purkinje cells has divergent effects on the motor behavior of juvenile animals: a compromise of rotating rod performance and a simultaneous enhancement of initial exploratory activity. With age, these animals develop incoordination with concomitant progressive Purkinje neuron dendritic and somatic atrophy but relatively little cell loss. Therefore, the eventual development of ataxia caused by the expression of a mutant SCA1 allele is not the result of cell death per se, but the result of cellular dysfunction and morphological alterations that occur before neuronal demise.

Type: Article
Title: Purkinje cell expression of a mutant SCA1 allele in transgenic mice leads to disparate effects on motor behaviours followed by a progressive cerebellar dysfunction and histological abnormalities
Open access status: An open access version is available from UCL Discovery
Language: English
Additional information: This work is licensed under the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported License. The license allows you to copy, distribute, and transmit the work, as well as adapting it. However, you must attribute the work to the author (but not in any way that suggests that they endorse you or your use of the work), and cannot use the work for commercial purposes without prior permission of the author. If you alter or build upon this work, you can distribute the resulting work only under the same or similar license to this one. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-sa/3.0/ or send a letter to Creative Commons, 444 Castro Street, Suite 900, Mountain View, California, 94041, USA.
Keywords: transgenic mice, neurodysfunction, CAG repeat, SCA1, Purkinje cell, motor behaviour
UCL classification: UCL
URI: https://discovery.ucl.ac.uk/id/eprint/184549
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