DNAJC5 and adult neuronal ceroid lipofuscinosis: It has been demonstrated that the two mutations causing Kufs disease/CLN4, Leu115Arg and Leu116Δ, localized at the cysteine string domain, interfere with normal CSPα/DNAJC5 palmitoylation (13, 26, 27) and lead to the formation of molecular accumulations.