The protective protein : a multifunctional lysosomal enzyme
1991
textabstractThis thesis describes the characterization of a lysosomal protein, the 'protective
protein', that has at least two functions. On the one hand it protects lysosomal ~galactosidase
and neuraminidase from degradation within the lysosome, hence its
name. On the other hand it has peptidase and deamidase activities, that could be
involved in protein turnover in lysosomes and hormone (in)activation. Degradation
is distinguished here from proteolytic processing, although both involve peptide
hydrolysis. The first is, however, an aspecific random process, carried out at multiple
sites, whereas the second is a highly specific (single) event. Given the putative
function of the protective protein it seemed appropriate to start with an overview of
intracellular sites of protein degradation, followed by a section on the biogenesis of
lysosomes. The introduction ends with a summary on what is known about lysosomal
storage disorders, a group of genetic diseases that are due to defects in lysosomal
proteins. The protective protein itself is impaired in the rare disorder galactosialidosis
and studies on this disease have been the basis for the discovery of the
protein and analysis of its functions.
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