Drosophila Models of Hereditary Spastic Paraplegia
© 2015 Elsevier Inc. All rights reserved. Hereditary spastic paraplegia (HSP) is a debilitating movement disorder with no prevention, no cure, and few options for symptom management. Rapid and relevant information is required, and the model organism Drosophila melanogaster can contribute significantly to our understanding of the cellular mechanisms underlying HSP pathology. We review HSP models based on fly orthologs of spastin, atlastin, reticulon-like 1, spichthyin, spartin, Kinesin heavy chain, swiss cheese, and unexamined causative loci. The relevance of these models to HSP is highlighted by their progressive neurodegeneration with distally increasing severity and impaired locomotor behaviors. Subcellularly, they implicate defects in synapse formation, microtubule regulation, endoplasmic reticulum morphogenesis, membrane trafficking, and axon transport. Much remains to be discovered from these models, including the relationship between these mechanisms. In addition, insights gained from the unique amenability of Drosophila to high-throughput genetic and in vivo pharmacological screens promise to advance our knowledge further and recommend potential therapeutic options.
Ozdowski, EF; Baxter, SL; Sherwood, NT
- Movement Disorders: Genetics and Models: Second Edition
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International Standard Book Number 13 (ISBN-13)
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