Drosophila Melanogaster Models of Motor Neuron Disease

Ruben J. Cauchi (Editor)
University of Malta, Msida, Malta

Series: Neurodegenerative Diseases – Laboratory and Clinical Research
BISAC: MED057000

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Volume 10

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Edited by I Leslie Rubin, Robert J Geller, Abby Mutic, Benjamin A Gitterman, Nathan Mutic, Wayne Garfinkel, Claire D Coles, Kurt Martinuzzi, and Joav Merrick

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Motor neuron diseases are the most catastrophic of neurodegenerative disorders. The cognitive function is spared, but the motor neuron degeneration translates into progressive muscle weakness and paralysis that propel the afflicted patient to eventual death. Neurodegenerative disorders constitute one of the major challenges of modern medicine in view of the current lack of effective therapies. The fruit fly, Drosophila melanogaster, has a distinguished history as an important model organism capable of shaping our fundamental understanding of life. Remarkably, the vast majority of all known human disease genes have a similar fly counterpart and at the molecular and physiological level, the basic principles of neuromuscular function are amazingly conserved between humans and Drosophila.

Combine this with the presence of numerous genetic tools developed over the last century allowing genes and the proteins they encode to be manipulated swiftly to decipher their in vivo function and you have a superb genetic animal model organism of disease. This publication singles out the past and recent accomplishments of Drosophila in modelling motor neuron disease including amyotrophic lateral sclerosis (Lou Gehrig’s disease), hereditary spastic paraplegias, Charcot-Marie-Tooth disease, spinal and bulbar muscular atrophy (Kennedy’s disease) and spinal muscular atrophy. The emphasis is on recent developments including the emerging molecular pathways underpinning these disorders. Genetic screens aimed at identifying novel genes that cause motor neuron degeneration or finding modifiers of the phenotype resulting from the disruption of disease-causative genes are also tackled. Importantly, this collection provides an inspiring look at the indispensability of the fruit fly, and of model organisms in general, to neuroscience research. (Imprint: Nova Biomedical )

Preface

Chapter 1: Genetics of Motor Neuron Disorders: From Gene Diversity to Common Cellular Conspirators in Selective Neuronal Killing;pp. 1-34
(Rebecca K. Sheean & Bradley J. Turner, Florey Institute of Neuroscience & Mental Health, University of Melbourne, Australia)

Chapter 2: A Secreted Ligand for Growth Cone Receptors, VAP Mediates the Cellular Pathological Defects in ALS;pp. 35-55
(Amina Moustaqim-Barrette, Mario Maira & Hiroshi Tsuda, Department of Neurology and Neurosurgery, McGill University, Canada)

Chapter 3: Flies in Motion: What Drosophila can tell us about Amyotrophic Lateral Sclerosis;pp. 57-83
(Andrees A. Morera, Alyssa Coyne & Daniela C. Zarnescu, Departments of Molecular and Cellular Biology, Neuroscience and Neurology, University of Arizona, Tucson, AZ, USA)

Chapter 4: Maintaining Long Supply Lines: Axon Degeneration and the Function of Hereditary Spastic Paraplegia Genes in Drosophila;pp. 85-120
(Belgin Yalçýn & Cahir J. O’Kane, Department of Genetics, University of Cambridge, UK)

Chapter 5: Drosophila as a Model for CMT Peripheral Neuropathy: Mutations in tRNA Synthetases as an Example;pp. 121-146
(Georg Steffes & Erik Storkebaum, Molecular Neurogenetics Laboratory, Max Planck Institute forMolecularBiomedicine,Muenster,Germany)
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Chapter 6: Lessons from Drosophila in Neurodegeneration: Mechanisms of Toxicity and Therapeutic Targets in Spinal and Bulbar Muscular Atrophy;pp. 147-170
(Adrienne M. Wang, Department of Pathology, University of Washington, Seattle, WA, USA)

Chapter 7: Spinal Muscular Atrophy: Insights from the Fruit Fly;pp. 171-184
(Stuart J. Grice, Kavita Praveen, A. Gregory Matera and Ji-Long Liu, MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, UK and others)

Chapter 8: Genetic Screens in Drosophila and their Application in Motor Neuron Disease Models;pp. 185-210
(Liya E. Jose, Patrik Verstreken & Sven Vilain, VIB Center for The Biology of Disease, Leuven, Belgium)

Index

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