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H-Asn(GlcNAc-β-D)-OH is a derivative of the amino acid L-asparagine (Asn; Item Nos. 35022 | 34519). During the catabolism of Asn-linked glycoproteins in the lysosome, H-Asn(GlcNAc-β-D)-OH is hydrolyzed via aspartylglucosaminidase to form Asn and N-acetylglucosamine (GlcNAc).1,2 Urinary levels of H-Asn(GlcNAc-β-D)-OH are increased in patients with aspartylglycosaminuria, a lysosomal storage disorder characterized by neurological deficits and skeletal abnormalities.2
WARNING This product is not for human or veterinary use.
1. Lysosomal degradation of Asn-
2. Aspartylglycosaminuria: Protein chemistry and molecular biology of the most common lysosomal storage disorder of glycoprotein degradation. The FASEB Journal 7(13), 1247-1256 (1993).