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NGLY1-CDDG is a congenital disorder of deglycosylation caused by a defective peptide:N-glycanase (PNG). To date, all… Expand Aspartylglucosaminuria (AGU), a recessively inherited lysosomal storage disease, is the most common disorder of glycoprotein… Expand SummaryAspartylglycosaminuria (AGU) is a hereditary metabolic disorder charaterized by slowly progressive mental deterioration… Expand Aspartylglycosaminuria (AGU), the most common lysosomal disorder of glycoprotein degradation, is caused by deficient activity of… Expand A sensitive method for quantitative analysis of aspartylglucosamine as its dabsyl chloride derivative by high-performance liquid… Expand An enzyme preparation from jack-bean meal hydrolyzed beta-aspartylglucosylamine linkages in glycopeptides. The enzyme could… Expand Two members of a consanguineous Italian family are described with the symptoms of aspartylglycosaminuria. Both patients exhibit… Expand A young woman with a previous history of anorexia nervosa presented with severe finger clubbing. Urine samples intermittently… Expand A mannose-containing sialooligosaccharide has been isolated from the urine of a patient with a newly recognized mucolipidosis… Expand Abstract Two-dimensional thin-layer chromatography of the urine samples of aspartylglucosaminuria patients showed the presence of… Expand