Sialic acid storage diseases. A multiple lysosomal transport defect for acidic monosaccharides.
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چکیده
منابع مشابه
Sialic acid storage diseases. A multiple lysosomal transport defect for acidic monosaccharides.
A defective efflux of free sialic acid from the lysosomal compartment has been found in the clinically heterogeneous group of sialic acid storage disorders. Using radiolabeled sialic acid (NeuAc) as a substrate, we have recently detected and characterized a proton-driven carrier for sialic acid in the lysosomal membrane from rat liver. This carrier also recognizes and transports other acidic mo...
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Sialic acid and glucuronic acid are monocarboxylated monosaccharides, which are normally present in sugar side chains of glycoproteins, glycolipids, and glycosaminoglycans. After degradation of these compounds in lysosomes, the free monosaccharides are released from the lysosome by a specific membrane transport system. This transport system is deficient in the human hereditary lysosomal sialic ...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1991
ISSN: 0021-9738
DOI: 10.1172/jci115136