Article
Disorders of glycoprotein degradation.
Journal of inherited metabolic disease - 1 Jan 1990
Cantz M, Ulrich-Bott B
Abstract excerpt
The intracellular degradation of glycoproteins occurs predominantly in the lysosomes through the concerted action of proteases and glycosidases. Genetic defects in any of the enzymes cleaving the oligosaccharide side chains lead to specific diseases because of an excessive lysosomal accumulation of partially degraded material, mostly oligosaccharides. This paper presents an overview of the biochemistry and the...
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