The model organism used in the O’Sullivan lab:
The O’Sullivan lab has opted to use the fruit fly Drosophila melanogaster in order to model HSP. A model organism in which there is a very well conserved homologue to ARL6IP1, CG101026 (Fowler and O’Sullivan 2016). The fruit fly has a number of advantages associated with it as a model organism as for one Drosophila melanogaster has a short life cycle, a rapid generation time and produce a large number of offspring meaning experiments can be performed quite quickly and fly stocks can be maintained inexpensively and easily. In regards to studying neurodegenerative disease specifically, the fruit fly Drosophila melanogaster possesses a well-established nervous system and although an invertebrate species, possess a ventral nerve cord, which is analogous to the spinal cord with motor neurons that connect from the ventral nerve cord and synapse onto muscles at the neuromuscular junction. Additionally Drosophila melanogaster is a genetically amenable model organism with a relatively small genome with one of the major advantages of using it as a model organism being the fact that
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With the exploitation of the pan neuronal driver nSyb-GAL4 investigation of the effect of Arl6IP1 knockdown specifically in neurons was enabled. It was found that Arl6IP1 knockdown specifically in neurons had no significant affect on the lifespan of the flies when compared to controls. However using a negative geotaxis assay to investigate the affect Arl6IP1 knockdown on locomotive behaviour it was discovered that the labs model of HSP displayed a progressive neurodegenerative phenotype (Fowler and O’Sullivan 2016). From these findings it was concluded that this model of HSP would prove to be an advantageous model going fourth to further investigate the cause of HSP at the cellular level as this model emulated some of the key characteristics of the human