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SHH Pathway and Cerebellar Development

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Abstract

The morphogenetic factor Sonic hedgehog (SHH) has been discovered as one of the masterplayers in cerebellar patterning and was subjected to intensive investigation during the last decade. During early postnatal development, this continuously secreted cholesterol-modified protein drives the expansion of the largest neuronal population of the brain, the granular cells. Moreover, it acts on Bergmann glia differentiation and would potentially affect Purkinje cells homeostasis at adult age. The cerebellar cortex constituted an ideal developmental model to dissect out the upstream mechanisms and downstream targets of this complex pathway. Its deep understanding discloses some of the mechanistic disorders underlying pediatric tumorigenesis, congenital ataxia, and mental retardation. Therapeutical use of its regulators has been consolidated on murine transgenic models and is now considered as a realistic human clinical application. Here, we will review the most recent advances made in the comprehensive understanding of SHH involvement in cerebellar development and pathology.

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Acknowledgments

Catherine Vaillant is supported by a research fellowship from the Swiss National foundation and Denis Monard by the Novartis Research Foundation. We would like to thank Dr Filippo Rijli for his critical reading of the manuscript.

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  1. Developmental Genetics, Department Biomedicine, University of Basel, Mattenstrasse 28, CH-4058, Basel, Switzerland

    Catherine Vaillant

  2. Friedrich Miescher Institut for Biomedical Research, Maubeerstrasse 66, CH-4058, Basel, Switzerland

    Denis Monard

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  1. Catherine Vaillant
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Correspondence to Denis Monard.

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Vaillant, C., Monard, D. SHH Pathway and Cerebellar Development. Cerebellum 8, 291–301 (2009). https://doi.org/10.1007/s12311-009-0094-8

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