Open AccessPrimary Structure of Three Mannosyl‐glycoasparagines and Nine Sialyl‐oligosaccharides Isolated from the Urine of Two Patients with Gaucher's Disease (Infantile Form)
Author(s) -
MICHALSKI JeanClaude,
MONTREUIL Jean,
STRECKER Gérard,
HALBEEK Herman,
DORLAND Lambertus,
VLIEGENTHART Johannes F. G.,
CARTIGNY Bernard,
FARRIAUX JeanPierre
Publication year - 1983
Publication title -
european journal of biochemistry
Language(s) - English
Resource type - Journals
eISSN - 1432-1033
pISSN - 0014-2956
DOI - 10.1111/j.1432-1033.1983.tb07373.x
Subject(s) - urine , glucocerebroside , mucolipidosis , spleen , disease , chemistry , carbohydrate , medicine , pathology , biochemistry , glucocerebrosidase , enzyme
The urine of two patients with the infantile form of Gauche's disease were analyzed for their carbohydrate content. Nine sialyl‐okigosaccharides were isolated and characterized. Compounds with identical structures occur in the urine of patient with sialidosis. Furthermore, an abnormal excretion of mannosyl‐glycoasparagines was observed. Three of thses compounds were isolated and identified:This carbohydrate material, which is found also in the spleen of the patients, probably originastes from obstruction of lysosomal functioning by the accumulation of an excessive amount of glucocerebroside.