Journal
BIOLOGICAL CHEMISTRY
Volume 383, Issue 5, Pages 751-756Publisher
WALTER DE GRUYTER GMBH
DOI: 10.1515/BC.2002.078
Keywords
cathepsin; lysosome; mannose 6-phosphate; mucolipidosis; processing; storage disease
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Proteolytic maturation of lysosomal proteinases is initiated after receptormediated targeting to prelysosomal compartments, while terminal processing occurs upon delivery to lysosomes. These late processing events are impaired in patients suffering from inherited lysosomal disorders, such as sialic acid storage disease and mucolipidosis II (Icell disease). Lysosomes in the affected cells display marked changes in their physiological and morphological properties, with features reminiscent of prelysosomal compartments. This indicates that the absence of mature lysosomes interferes with the final processing steps during the biosynthesis of lysosomal proteinases. Thus, impaired proteinase maturation reflects an incompetent lysosomal apparatus and as such can be seen as a hallmark of lysosomal storage diseases.
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