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Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome

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Modeling Fragile X Syndrome

Part of the book series: Results and Problems in Cell Differentiation ((RESULTS,volume 54))

Abstract

The Drosophila genome contains most genes known to be involved in heritable disease. The extraordinary genetic malleability of Drosophila, coupled to sophisticated imaging, electrophysiology, and behavioral paradigms, has paved the way for insightful mechanistic studies on the causes of developmental and neurological disease as well as many possible interventions. Here, we focus on one of the most advanced examples of Drosophila genetic disease modeling, the Drosophila model of Fragile X Syndrome, which for the past decade has provided key advances into the molecular, cellular, and behavioral defects underlying this devastating disorder. We discuss the multitude of RNAs and proteins that interact with the disease-causing FMR1 gene product, whose function is conserved from Drosophila to human. In turn, we consider FMR1 mechanistic relationships in non-neuronal tissues (germ cells and embryos), peripheral motor and sensory circuits, and central brain circuits involved in circadian clock activity and learning/memory.

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  1. Indiana University School of Medicine, 1234 Notre Dame Avenue, South Bend, IN, 46617, USA

    Charles R. Tessier

  2. Departments of Biological Sciences and Cell and Developmental Biology, Kennedy Center for Research on Human Development, Vanderbilt University, Station B, 6270A MRB III, 465 21st Avenue South, Nashville, TN, 37232, USA

    Kendal Broadie

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  1. New York State Institute for Basic Resea, Forest Hill Road 1050, Staten Island, 10314, USA

    Robert B. Denman

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Tessier, C.R., Broadie, K. (2012). Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome. In: Denman, R. (eds) Modeling Fragile X Syndrome. Results and Problems in Cell Differentiation, vol 54. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-21649-7_7

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