Neurodegenerative diseases (NDs) encompass a heterogeneous group of disorders that result in neuronal degeneration, leading to physical and cognitive decline and ultimately death. Although NDs have been the focus of intense research efforts, they are poorly understood, largely untreatable and represent a huge global health and economic challenge within an aging population. Stress granules (SGs) are membrane-less organelles comprised of mRNA and protein that modulate signalling pathways in response to stress, promoting cell survival. Links between SG dysfunction and human disease including NDs are well established. This thesis aimed to characterise SG properties and dynamics in iPSC-derived models of Alzheimer’s disease (AD), Amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and a HeLa model of CLN3 Batten disease (BD), a cause of juvenile neurodegeneration. By investigating the properties and dynamics of SGs we hoped to understand commonly deregulated pathways which would advance shared understanding of how SGs drive pathogenesis in NDs. In chapters 3 and 4, we show the characterisation of neuronal precursor cell (NPC) models, techniques for SG visualisation and assessment of their clearance dynamics, as well as quantification of translation efficiency. We show aberrant SG properties and alter removal dynamics, as well as translational defects are associated with mutations causative of ALS/FTD and AD. Chapter 5 shows the optimisation of assays used in preceding chapters in terminally differentiated neurons. Section 6 is adapted from a recent publication (Relton et al, 2023). In this study we show SG dysfunction is also associated with loss of BD related gene CLN3. As interest grows into the use of SG modulating drugs to alleviate NDs pathology, this work uncovers dysregulated SG biology across a range of novel ND models. These findings will aid further knowledge in the field of SGs and disease, with the ultimate goal of discovering new targets for therapeutic development.