Abstract
AbstractPalmitoyl-protein thioesterase (PPT) is a lysosomal enzyme that removes fatty acids bound in thioester linkage to cysteine residues in fatty acylated proteins (1–4). Mutations in the PPT gene that affect enzyme activity cause a recessively inherited lysosomal storage disorder, infantile neuronal ceroid lipofuscinosis (INCL) (5). The disorder is characterized by severe central nervous system degeneration and lysosomal inclusions in the form of granular osmiophilic deposits in many tissues (6,7).KeywordsMicrofuge TubeLysosomal Storage DisorderNeuronal Ceroid LipofuscinosisChemical HydrolysisAffect Enzyme ActivityThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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