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The viability staining was performed with the following reagents: Zombie Violet Fixable Viability Dye (1:1000, #423113, BioLegend) and Annexin V-APC (1:100, #550474, BD Biosciences

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The deficiency in activity may come about via a total absence of enzyme protein caused by a mutation of the nuclear gene that produces no mRNA or produces mRNA leading to a defective or unstable enzyme, via a defective post-translational modification of the lysosomal enzyme, or via the absence of a specific activator protein required by some enzymes for the initiation of activity