The toppler mouse: a novel mutant exhibiting loss of Purkinje cells

The Journal of Comparative Neurology
Cynthia S DuchalaWendy B Macklin

Abstract

We describe the genetic and neurological features of toppler, a spontaneous autosomal mutation that appeared in a colony of FVB/N mice and that manifests as severe ataxia appearing at around 12 days of age, worsening with age. The lifespan of affected mice is 8-12 months, with occasional mice living longer. Both homozygous males and females are fertile, and females are able to nurture litters. Histological examination of brain revealed no striking abnormalities other than the loss of cerebellar Purkinje cells. The toppler mutation was mapped to mouse chromosome 8, and to assess whether it was novel or a recurrence of a previously described chromosome 8 mouse mutant, toppler mice were crossed with the nervous and tottering mouse mutants. These studies demonstrate that toppler is a unique mouse mutation. Purkinje cell abnormalities in toppler mice were obvious around postnatal day (P) 14, i.e., toppler Purkinje cells already exhibited abnormal morphology. Staining for calbindin, a calcium binding protein enriched in Purkinje cells, showed altered dendritic morphology. Between P14 and P30, dramatic Purkinje cell loss occurred, although there were differences in the degree of Purkinje cell loss in each lobule. At P30, the surviving...Continue Reading

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Citations

May 9, 2009·The Cerebellum·Kejian ChenTodd W Preston
Nov 26, 2010·The Cerebellum·Carol L ArmstrongMichael William Vogel
Mar 4, 2009·Brain Research·Sreenivasulu ChintalaRichard T Swank
Feb 21, 2009·Lab Animal·Risa Pesapane, Deborah J Good
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Jul 21, 2005·Theoretical Biology & Medical Modelling·Skirmantas Janusonis
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Jun 8, 2011·The European Journal of Neuroscience·Justyna R Sarna, Richard Hawkes
Dec 18, 2015·Physiological Research·F VoŽeh

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