Neuronal migration defect of the developing cerebellar vermis in substrains of C57Bl/6 mice: Cytoarchitecture and prevalence of molecular layer heterotopia

Zareema Mangaru, Elsaid Salem, Matthew Sherman, Sarah E. Van Dine, Ankur Bhambri, Joshua C. Brumberg, Eric K. Richfield, Lisa A. Gabel, Raddy L. Ramos

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Abstract

Abnormal development of the cerebellum is often associated with disorders of movement, postural control, and motor learning. Rodent models are widely used to study normal and abnormal cerebellar development and have revealed the roles of many important genetic and environmental factors. In the present report we describe the prevalence and cytoarchitecture of molecular-layer heterotopia, a malformation of neuronal migration, in the cerebellar vermis of C57BL/6 mice and closely-related strains. In particular, we found a diverse number of cell-types affected by these malformations including Purkinje cells, granule cells, inhibitory interneurons (GABAergic and glycinergic), and glia. Heterotopia were not observed in a sample of wild-derived mice, outbred mice, or inbred mice not closely related to C57BL/6 mice. These data are relevant to the use of C57BL/6 mice as models in the study of brain and behavior relationships and provide greater understanding of human cerebellar dysplasia.

Original languageEnglish (US)
Pages (from-to)28-39
Number of pages12
JournalDevelopmental Neuroscience
Volume35
Issue number1
DOIs
StatePublished - Jan 1 2013
Externally publishedYes

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Keywords

  • Cerebellar dysplasia
  • Ectopia
  • Foliation
  • Lamination
  • Malformation

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