Palmitoylation-induced aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosis

Specific mutations in the chaperone protein CSPα cause adult-onset neuronal ceroid lipofuscinosis. Results: These mutants form SDS-resistant aggregates in a palmitoylation-dependent manner in cell lines and brain samples from mutation carriers. Conclusion: Palmitoylation induces disease-causing CSPα mutants to form SDS-resistant aggregates. Significance: Formation of SDS-resistant CSPα aggregates may underlie development of adult-onset neuronal ceroid lipofuscinosis.