Abstract

AbstractMost known lysosomal enzymes are soluble proteins that are localized in the lysosomal matrix. After biosynthesis, these proteins are targeted to lysosomes by a combination of mechanisms operating to different extents in different tissues. This allows for finely and individually tuning the proportion of lysosomal targeting and secretion. A detailed knowledge is available on the synthesis of mannose-6-phosphate (M6P)-recognition markers on soluble lysosomal enzymes and their M6P-dependent targeting as well as on sorting of M6P receptors and several proteins associated with lysosomal membranes. In humans, defects in the synthesis of M6P residues in lysosomal enzymes present as mucolipidosis (ML) II, in which the phosphorylation is missing completely, and as its milder form, ML III, in which a residual phosphorylation is present. This chapter focuses on the synthesis and sorting of the M6P-bearing soluble lysosomal enzymes.

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