Abstract: Lysosomal storage disorders and their impact upon the central nervous system: Lysosomal storage disorders (LSDs) are a group of over 70 rare inherited metabolic disorders (Platt et al., 2018). They are caused by dysfunction of lysosomes, organelles that contain enzymes responsible for digesting macromolecules. In functional lysosomes, these enzymes break down complex substrates, and the resulting fragments are recycled. Individual LSDs are caused by mutations in genes that encode lysosomal enzymes or other proteins crucial for lysosome function (Platt et al., 2018). These mutations cause lysosomal dysfunction and intralysosomal accumulation of undigested substances. This accumulation of “storage material” does not necessarily directly damage cells, but other poorly understood consequences of lysosomal dysfunction impair cell health over time. The vast majority of LSDs are inherited in an autosomal recessive or X-linked recessive manner (Platt et al., 2018). Although individually rare, the overall prevalence of LSDs is estimated at 1 in 5000 live births. Symptoms of LSDs are diverse and progressive, significantly affecting quality of life of patients, and ultimately proving fatal (Platt et al., 2018). Approximately two thirds of LSDs have severe effects on the central nervous system (CNS) characterized by progressive neurodegeneration (Platt et al., 2018). Research into these “neuronopathic” LSDs has primarily focused on the CNS, but there is increasing evidence that these LSDs also damage the peripheral nervous system, including recently demonstrated damage to the enteric nervous system (ENS).