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Drosophila Melanogaster Models of Motor Neuron Disease

Drosophila Melanogaster Models of Motor Neuron Disease

9781626187474
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Description
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 Gehrigs disease), hereditary spastic paraplegias, Charcot-Marie-Tooth disease, spinal and bulbar muscular atrophy (Kennedys 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.
Product Details
74229
9781626187474
9781626187474

Data sheet

Publication date
2013
Issue number
1
Cover
hard cover
Pages count
245
Dimensions (mm)
180.00 x 260.00
Weight (g)
652
  • Preface; Genetics of Motor Neuron Disorders:: From Gene Diversity to Common Cellular Conspirators in Selective Neuronal Killing; A Secreted Ligand for Growth Cone Receptors, VAP Mediates the Cellular Pathological Defects in ALS; Flies in Motion:: What Drosophila can tell us about Amyotrophic Lateral Sclerosis; Maintaining Long Supply Lines:: Axon Degeneration & the Function of Hereditary Spastic Paraplegia Genes in Drosophila; Drosophila as a Model for CMT Peripheral Neuropathy:: Mutations in tRNA Synthetases as an Example; Lessons from Drosophila in Neurodegeneration:: Mechanisms of Toxicity & Therapeutic Targets in Spinal & Bulbar Muscular Atrophy; Spinal Muscular Atrophy:: Insights from the Fruit Fly; Genetic Screens in Drosophila & their Application in Motor Neuron Disease Models; Index.
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