TY  - BOOK
AU  - Cauchi,Ruben J.
TI  - Drosophila melanogaster models of motor neuron disease 
T2  - Neurodegenerative diseases-laboratory and clinical research
SN  - 9781626187979
PY  - 2013///]
CY  - New York
PB  - Nova Biomedical
KW  - Amyotrophic lateral sclerosis
KW  - Motor neurons
KW  - Models
KW  - Drosophila melanogaster
N1  - Includes bibliographical references and index; DROSOPHILA MELANOGASTER MODELS OF MOTOR NEURON DISEASE ; DROSOPHILA MELANOGASTER MODELS OF MOTOR NEURON DISEASE ; Library of Congress Cataloging-in-Publication Data ; CONTENTS ; PREFACE ; A RESPONSIBLE CHOICE OF MODEL ORGANISM ; Chapter 1 GENETICS OF MOTOR NEURON DISORDERS: FROM GENE DIVERSITY TO COMMON CELLULAR CONSPIRATORS IN SELECTIVE NEURONAL KILLING ; ABSTRACT ; ABBREVIATIONS ; INTRODUCTION ; 1. AMYOTROPHIC LATERAL SCLEROSIS ; 1.1. Common Pathogenic Mechanisms in ALS ; 1.1.1. Oxidative Stress and Mitochondrial Dysfunction; 1.1.2. Dysregulated Intracellular Membrane Trafficking; 1.1.3. Abnormal Axonal Transport and Cytoskeleton 1.1.4. Defective RNA Processing ; 1.1.5. Impaired proteostasis ; 2. HEREDITARY SPASTIC PARAPLEGIA ; 2.1. Common Pathogenic Mechanisms in HSP ; 2.1.1. Oxidative Stress and Mitochondrial Dysfunction ; 2.1.2. Dysregulated Intracellular Membrane Trafficking ; 2.1.3. Abnormal Axonal Transport and Cytoskeleton; 2.1.4. Defective Myelination and Lipid Metabolism; 3. CHARCOT-MARIE-TOOTH DISEASE ; 3.1. Common Pathogenic Mechanisms in CMT2 ; 3.1.1. Oxidative Stress and Mitochondrial Dysfunction ; 3.1.2. Dysregulated Intracellular Membrane Trafficking; 3.1.3. Abnormal Axonal Transport and Cytoskeleton 3.1.4. Defective Myelination and Lipid Metabolism ; 3.1.5. Impaired proteostasis ; 5. SPINAL MUSCULAR ATROPHIES AND OTHER MOTOR NEURON DISORDERS ; 5.1. Common Pathogenic Mechanisms in SMA and Other MNDs ; 5.1.1. Dysregulated Intracellular Membrane Trafficking ; 5.1.2. Abnormal Axonal Transport and Cytoskeleton ; 5.1.3. Defective RNA Processing ; 5.1.4. Impaired Proteostasis ; CONCLUSION ; ACKNOWLEDGMENTS ; ABOUT THE AUTHORS ; REFERENCES; Chapter 2 A SECRETED LIGAND FOR GROWTH CONE RECEPTORS, VAP MEDIATES THE CELLULAR PATHOLOGICAL DEFECTS IN ALS ABSTRACT; ABBREVIATIONS ; INTRODUCTION ; STUDIES ON MUTATIONS ASSOCIATED WITH FAMILIAL FORMS OF ALS ENABLE US TO UNDERSTAND THE CORE PATHOLOGY OF ALS ; DOMINANTLY INHERITED MISSENSE MUTATIONS IN VAP ARE ASSOCIATED WITH A FAMILIAL FORM OF ALS ; VAP IS AN ER TRANSMEMBRANE PROTEIN CONTAINING A MSP DOMAIN ; VAP FUNCTIONS IN NOVEL SIGNALLING PATHWAYS ; CLEAVAGE OF VAP RELEASES MSP DOMAIN-CONTAINING FRAGMENTS THAT ARE SECRETED; VAP ACTS AS A LIGAND FOR EPH RECEPTOR AND MEDIATES EPH SIGNALLING VAP MSP MEDIATES ROBO AND LAR RECEPTORS AND FUNCTIONS IN THE MAINTENANCE OF THE MITOCHONDRIA IN MUSCLE ; ALS MUTATION CAUSES TWO DIFFERENT TYPES OF DEFECTS: A FAILURE OF VAP SECRETION (LOSS-OF-FUNCTION) AND ER STRESS (GAIN-OF-FUNCTION) AND ER STRESS (GAIN-OF-FUNCTION OR TOXIC DEFECTS) ; CONCLUSION ; ABOUT THE AUTHORS ; REFERENCES ; Chapter 3 FLIES IN MOTION: WHAT DROSOPHILA CAN TELL US ABOUT AMYOTROPHIC LATERAL SCLEROSIS ; ABSTRACT ; INTRODUCTION ; ALS IN MODEL ORGANISMS: YEAST TO MICE ; Current Hypotheses for ALS
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