Dysfunction of GABAergic neurons in the adult ventral nerve cord underlies SCA3- associated tremor in Drosophila melanogaster

Despite its prevalence, its underlying mechanisms are not well understood. We previously examined motor defects in a Drosophila model of Machado Joseph disease (MJD), also known as Spinocerebellar ataxia 3 (SCA3).

We found that the SCA3 fly model exhibits distinct gait and tremor signatures analogous to human SCA3 motor dysfunctions, and can hence be used to investigate the neurogenetic mechanisms of SCA3 tremor1. Here, we identify and characterise the neuronal populations involved in SCA3-associated tremor. Through a genetic screen, we identified a population of neurons in the ventral nerve cord (VNC) whose dysfunction is sufficient to generate tremor.