title: The core-specific lysosomal alpha(1-6)-mannosidase activity depends on aspartamidohydrolase activity.
abstract: The substrate specificity of the core-specific rat liver lysosomal alpha(1-6)-mannosidase was investigated using mannosylated oligosaccharides and glycoasparagines. Hydrolysis of Man(alpha 1-6) linkage hydrolysis was demonstrated to follow the action of endoglycosidases, namely aspartyl-N-acetyl-beta-D-glucosaminidase and endo-N-acetyl-beta-D-glucosaminidase. The results are discussed with respect to the nature of the carbohydrate materials stored in the tissues and excreted in the urine from patients suffering from aspartylglucosaminuria and fucosidosis.
authors.affiliation: Unité Mixte de Recherche du CNRS no. 111, Université des Sciences et Technologies de Lille, Villeneuve d'Ascq, France.
authors.forename: J F
authors.initials: JF
authors.lastname: Haeuw
authors.forename: T
authors.initials: T
authors.lastname: Grard
authors.forename: C
authors.initials: C
authors.lastname: Alonso
authors.forename: G
authors.initials: G
authors.lastname: Strecker
authors.forename: J C
authors.initials: JC
authors.lastname: Michalski
mesh_terms: D000107:Acetylation; D000818:Animals; D001227:Aspartylglucosylaminase / Q000378:metabolism; D002240:Carbohydrate Sequence; D006020:Glycopeptides / Q000378:metabolism; D008247:Lysosomes / Q000201:enzymology*; D008361:Mannosidases / Q000378:metabolism*; D013058:Mass Spectrometry; D008969:Molecular Sequence Data; D009844:Oligosaccharides / Q000378:metabolism; D051381:Rats; D013379:Substrate Specificity
chemical_list: D006020:Glycopeptides; D009844:Oligosaccharides; C007872:glycoasparagines; D008361:Mannosidases; C078166:alpha(1-6)mannosidase; D001227:Aspartylglucosylaminase