The
neuronal ceroid lipofuscinoses (NCLs), collectively referred to as
Batten disease, are a group of fatal
neurodegenerative disorders that primarily affect children. The etiology of
Batten disease is linked to mutations in 13 genes that encode distinct CLN
proteins, whose functions have yet to be fully elucidated. The social amoeba Dictyostelium discoideum has been adopted as an efficient and powerful model system for studying the diverse cellular roles of CLN
proteins. The genome of D. discoideum encodes several homologs of human CLN
proteins, and a growing body of literature supports the conserved roles and networking of CLN
proteins in D. discoideum and humans. In humans, CLN
proteins have diverse cellular roles related to autophagy, signal transduction,
lipid homeostasis, lysosomal ion homeostasis, and intracellular trafficking. Recent work also indicates that CLN
proteins play an important role in
protein secretion. Remarkably, many of these findings have found parallels in studies with D. discoideum. Accordingly, this review will highlight the translatable value of novel work with D. discoideum in the field of NCL research and propose further avenues of research using this biomedical model organism for studying the NCLs.