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Gewählte Publikation:

Mach, L.
(2002): Biosynthesis of lysosomal proteinases in health and disease.
Biol Chem. 2002; 383(5):751-756 FullText FullText_BOKU

Abstract:
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.
Autor*innen der BOKU Wien:
Mach Lukas
Find related publications in this database (using NML MeSH Indexing)
Animals -
Carbohydrate Metabolism, Inborn Errors - metabolism
Cathepsins - biosynthesis
Cells, Cultured - biosynthesis
Fibroblasts - metabolism
Humans - metabolism
Lysosomal Storage Diseases - enzymology
Lysosomes - enzymology
Mice - enzymology
Protein Processing, Post-Translational - enzymology

Find related publications in this database (Keywords)
cathepsin
lysosome
mannose 6-phosphate
mucolipidosis
processing
storage disease


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