We have identified a DEAD box RNA helicase and christened it as maheshvara after the powerful Hindu God Lord Shiva. Maheshvara is a novel modulator of important signaling pathways like Notch and JAK/STAT. This novel gene has an orthologue of human DDX59. Our study parallels our identification of DDX59 mutations in the human orthologue of maheshvara which causes neurological defects in humans. Our observations suggests the role of maheshvara (mahe) in modulating cellular signaling, neuronal development and disease.In addition, We have also identified novel RNA binding proteins (RBPs) which suppresses Spinocerebellar Ataxia 8 (SCA8) disease pathology in Drosophila. Among these RBPs is Spoonbill and its interacting partners dSIP and Survivor Motor Neuron Protein (Smn) which suppress the neurodegenerative features associated with SCA8. Using our SCA8 Drosophila model we have also observed that specific RBPs like Spoon and dSIP are abnormally recruited into the toxic SCA8 RNA foci and this results in their depletion from the physiological pool. Ectopic expression of these vital RBPs thus restores the perturbed cellular machinery and reduce the pathological features associated with SCA8. These interesting observations have guided us to believe that multiple neurodevelopmental disorders with unlinked disease etiology may share overlapping molecular mechanism and cause disease pathogenesis and this may prove to be instrumental in identifying common therapeutic and diagnostic interventions. Thus, Drosophila not only provides a platform for untangling complex conserved cellular pathways but provides us interesting insight into the molecular mechanisms of the of neurodegenerative diseases.