Drosophila Melanogaster Models Of Motor Neuron Disease

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.

Dedicated to our readers, we include novel information (not reported in IntechOpen’s books before) about new contributions of aberrant astrocytes to MND damage and death in the SOD1G93A rat experimental model of ALS; novel genetic studies on ALS; an update of the structural and functional consequences of the spinal muscular atrophy-linked mutations of the survival motor neuron protein; stem cell therapy for MND; and the novel treatment for SMA and ALS in the introductory chapter. This book contains selected peer-reviewed chapters written by international researchers. In this publication, the readers will find a compilation of state-of-the-art reviews about etiology, therapies, investigations, the molecular basis of disease progression and clinical manifestations, and the genetic familial ALS, as well as novel therapeutic modalities. We look forward with confidence and pride to the remarkable role that this book will play for a new vision and mission.

This book contains 12 chapters divided into two sections. Section 1 is "Drosophila - Model for Genetics." It covers introduction, chromosomal polymorphism, polytene chromosomes, chromosomal inversion, chromosomal evolution, cell cycle regulators in meiosis and nongenetic transgenerational inheritance in Drosophila. It also includes ecological genetics, wild-type strains, morphometric analysis, cytostatics, frequencies of early and late embryonic lethals (EEL and LEL) and mosaic imaginal discs of Drosophila for genetic analysis in biomedical research. Section 2 is "Drosophila - Model for Therapeutics." It explains Drosophila as model for human diseases, neurodegeneration, heart-kidney metabolic disorders, cancer, pathophysiology of Parkinson's disease, dopamine, neuroprotective therapeutics, mitochondrial dysfunction and translational research. It also covers Drosophila role in ubiquitin-carboxyl-terminal hydrolase-L1 (UCH-L1) protein, eye development, anti-dUCH antibody, neuropathy target esterase (NTE), organophosphorous compound-induced delayed neuropathy (OPIDN) and hereditary spastic paraplegia (HSP). It also includes substrate specificities, kinetic parameters of recombinant glutathione S-transferases E6 and E7 (DmGSTE6 and DmGSTE7), detoxification and insecticidal resistance and antiviral immunity in Drosophila.

'Motor Neuron Disease in Adults' reviews new information from 1998 as it applies to all aspects of motor neuron disease. Articles included use evidence-based methods to ensure that the new information is solid and advances the topic. The book can be used by anyone who provides any type of care to ALS patients.

Most biological pathways, physical and neurological properties are highly conserved between humans and Drosophila and nearly 75% of human disease-causing genes have a functional homologue in Drosophila. This volume provides recent advances in Drosophila models for various human diseases, with each chapter providing a review of studies involving Drosophila models, as well as detailed protocols commonly used in laboratories. Starting with a review of Drosophila’s value as a highly tractable model organism for studying human diseases, subsequent chapters present Drosophila models for specific human diseases. The book provides a useful resource for all scientists who are starting to use the Drosophila model in their studies, and for researchers working in the pharmaceutical industry and using new screening models to develop new medicines for various diseases.

Motor Neuron Disease: New Insights for the Healthcare Professional / 2012 Edition is a ScholarlyBrief™ that delivers timely, authoritative, comprehensive, and specialized information about Motor Neuron Disease in a concise format. The editors have built Motor Neuron Disease: New Insights for the Healthcare Professional / 2012 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Motor Neuron Disease in this eBook to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Motor Neuron Disease: New Insights for the Healthcare Professional / 2012 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.

Molecular and Cellular Therapies for Motor Neuron Diseases discusses the basics of the diseases, also covering advances in research and clinical trials. The book provides a resource for students that will help them learn the basics in a detailed manner that is required for scientists and clinicians. Users will find a comprehensive overview of the background of Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig’s Disease) and Spinal Muscular Atrophy (SMA), along with the current understanding of their genetics and mechanisms. In addition, the book details gene and cell therapies that have been developed and their translation to clinical trials. Provides an overview of gene and cell therapies for amyotrophic lateral sclerosis (ALS) and other motor neuron diseases Edited by a leading Neurosurgeon and two research scientists to promote synthesis between basic neuroscience and clinical relevance Presents a great resource for researchers and practitioners in neuroscience, neurology, and gene and cell therapy