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doi: 10.1038/sj.tpj.6500220

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Loss-of-function mutations of the palmitoyl-protein thioesterase 1 gene ( PPT ) that cause a severe neurodegenerative LSD, called CLN1 neuronal ceroid lipofuscinosis (NCL), impair maturation of V0a1 and other Ppt -modified proteins, leading to deficient assembly and activity of lysosomal v-ATPase 145