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Glycoprotein storage disease

Schindler disease (also called lysosomal a-N-acetylgalactosaminidase [a-NAGA] deficiency, Schindler Type) is 1 of the over 40 glycoprotein storage diseases. [Pg.39]

Humbel R, Collart M (1975) Oligosaccharides in the urine of patients with glycoprotein storage diseases. I. Rapid detection by thin-layer chromatography. Clin Chim Acta 60 143-145... [Pg.332]

Humans and animals are affected by genetic diseases that occur as a result of inhibition of glycoprotein-processing enzymes (reviewed by James et al., 2004). These diseases are collectively known as lysosomal storage diseases. The animal diseases, genetic or induced, have counterparts in humans. For example ... [Pg.46]

Golgi a-l)-mannosidase II lysosomal mannosidase glycoprotein A-linked oligosaccharide processing [toxic, neurotoxic effects mimic hereditary lysosomal storage disease mannosidosis] (3-D-Glucuronidase cx-1-iduronidase Glucosidase... [Pg.527]

Mucolipidoses Lysosomal storage diseases (see) arising from defects in glycoprotein biosynthesis. [Pg.412]

Degradation of A -linked glycoproteins completely to amino acids and monosaccharides is necessary to avoid the storage diseases mentioned above that have severe consequences for cells and tissues. This catabolism has been determined to occur in two major parts [9, 10] ... [Pg.1586]

Mendla, K. Baumkotter, J. Rosenau, C. Ulrich-Bott, B. Cantz, M. Defective Lysosomal Release of Glycoprotein-Derived Sialic Acid in Fibroblasts from Patients with Sialic Acid Storage Disease. Biochem. J. 1988, 250, 261-267. [Pg.1593]

See other pages where Glycoprotein storage disease is mentioned: [Pg.331]    [Pg.168]    [Pg.170]    [Pg.483]    [Pg.37]    [Pg.331]    [Pg.168]    [Pg.170]    [Pg.483]    [Pg.37]    [Pg.102]    [Pg.6]    [Pg.45]    [Pg.478]    [Pg.285]    [Pg.129]    [Pg.1952]    [Pg.252]    [Pg.1788]    [Pg.187]    [Pg.319]    [Pg.249]    [Pg.240]    [Pg.252]    [Pg.328]    [Pg.168]    [Pg.363]    [Pg.37]    [Pg.314]    [Pg.118]    [Pg.1210]    [Pg.1210]    [Pg.1580]    [Pg.1585]    [Pg.1589]    [Pg.2037]    [Pg.2038]    [Pg.2039]    [Pg.2045]   
See also in sourсe #XX -- [ Pg.331 ]



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