Disease-related phenotypes in a Drosophila model of hereditary spastic paraplegia are ameliorated by treatment with vinblastine
J. Clin. Invest. Genny Orso, et al. 115:3026 doi:10.1172/JCI24694 [Go to this article.]

Figure 1
Behavioral phenotypes caused by neuron-specific expression of Dspastin RNAi and the Dspastin K467R pathogenic mutant are efficiently antagonized by pharmacological treatment with vinblastine. (A and B) Neuronal transgenic expression of Dspastin RNAi and Dspastin K467R reduced the longevity of adult flies (A) and severely impaired adult locomotor performance (B), causing premature loss of climbing ability. Vinblastine treatment increased adult lifespan (A) and improved locomotor ability (B), as judged by the enhanced performance in the climbing assay, of flies expressing Dspastin RNAi and Dspastin K467R in neurons. Control genotypes were as follows: Elav-Gal4/+, UAS-Dspastin-RNAi/+, and UAS-Dspastin-K467R/+. Experimental genotypes were as follows: Elav-Gal4/+;UAS-Dspastin-RNAi/+ and Elav-Gal4/+;UAS-Dspastin-K467R/+.