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Aspartylglucosaminuria

what is Aspartylglucosaminuria source(wikipedia)

Aspartylglucosaminuria (AGU), also called aspartylglycosaminuria, is a rare, autosomal recessive lysosomal storage disorder caused by deficient activity of the enzyme N-aspartyl-beta-glucosaminidase (aspartylglucosaminidase). This enzyme normally cleaves long sugar chains known as oligosaccharides in the lysosome. When N-aspartyl-beta-glucosaminidase is deficient these long sugar chains build up and eventually lead to the clinical features of AGU. AGU is one of seven identified Glycoprotein Storage

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