Metabolic Adaptations to Interrupted Glycosaminoglycan Recycling

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Metabolic adaptations to interrupted glycosaminoglycan recycling.

Lysosomal storage diseases (LSD) are metabolic disorders characterized by accumulation of undegraded material. The mucopolysaccharidoses (MPS) are LSDs defined by the storage of glycosaminoglycans. Previously, we hypothesized that cells affected with LSD have increased energy expenditure for biosynthesis because of deficiencies of raw materials sequestered within the lysosome. Thus, LSDs can be...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 2009

ISSN: 0021-9258

DOI: 10.1074/jbc.m109.020818