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Movement Disorders: Genetics and Models: Second Edition

Drosophila Models of Hereditary Spastic Paraplegia

Publication ,  Chapter
Ozdowski, EF; Baxter, SL; Sherwood, NT
January 1, 2015

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.

Duke Scholars

DOI

ISBN

9780124051959

Publication Date

January 1, 2015

Start / End Page

1103 / 1122
 

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Ozdowski, E. F., Baxter, S. L., & Sherwood, N. T. (2015). Drosophila Models of Hereditary Spastic Paraplegia. In Movement Disorders: Genetics and Models: Second Edition (pp. 1103–1122). https://doi.org/10.1016/B978-0-12-405195-9.00073-1
Ozdowski, E. F., S. L. Baxter, and N. T. Sherwood. “Drosophila Models of Hereditary Spastic Paraplegia.” In Movement Disorders: Genetics and Models: Second Edition, 1103–22, 2015. https://doi.org/10.1016/B978-0-12-405195-9.00073-1.
Ozdowski EF, Baxter SL, Sherwood NT. Drosophila Models of Hereditary Spastic Paraplegia. In: Movement Disorders: Genetics and Models: Second Edition. 2015. p. 1103–22.
Ozdowski, E. F., et al. “Drosophila Models of Hereditary Spastic Paraplegia.” Movement Disorders: Genetics and Models: Second Edition, 2015, pp. 1103–22. Scopus, doi:10.1016/B978-0-12-405195-9.00073-1.
Ozdowski EF, Baxter SL, Sherwood NT. Drosophila Models of Hereditary Spastic Paraplegia. Movement Disorders: Genetics and Models: Second Edition. 2015. p. 1103–1122.
Journal cover image

DOI

ISBN

9780124051959

Publication Date

January 1, 2015

Start / End Page

1103 / 1122