ABSTRACT
Acid GAG were isolated from hepatic tissue from 3 patients with Hurler syndrome and 3 normal controls. Gross elevations in the uronic acid and hexosamine contents were found in Hurler livers compared with the normal ones. The total GAG concentration was significantly increased (about 25fold) in Hurler patients.
Subject(s)
Glycosaminoglycans/metabolism , Liver/metabolism , Mucopolysaccharidosis I/metabolism , Child, Preschool , Glycosaminoglycans/analysis , Hexosamines/analysis , Humans , Liver/analysis , Male , Uronic Acids/analysisABSTRACT
The isolation and characterization of glycosaminoglycans was performed in uterus and submaxillary glands of castrated female rats receiving daily doses of 20 or 200 mug of acetoxyprogesterone and 100 or 1000 mug of progesterone. Chromatographic separation of glycosaminoglycans was performed on cellulose microcolumns. The uronic acid concentration in the uterus was decreased by acetoxyprogesterone, in every chromatographic fraction, but not by progesterone. The uronic acid concentration in the submaxillary glands was decreased by acetoxyprogesterone in fractions 1 (glycoporteins) and 2 (hyaluronic acid) and by progesterone in fractions 1 (glycoproteins), 2 (hyaluronic acid) and 7 (heparin);
Subject(s)
Glycosaminoglycans/metabolism , Hydroxyprogesterones/pharmacology , Submandibular Gland/metabolism , Uterus/metabolism , Animals , Castration , Female , Organ Size/drug effects , Progesterone/pharmacology , Rats , Submandibular Gland/drug effects , Uronic Acids/metabolism , Uterus/drug effectsABSTRACT
Urine specimens from healthy children were concentrated and filtered through Sephadex G-100 and G-25. The eluate was recovered in fractions of 5 ml each. Total hexoses, galactose, total proteins and total uronic acids were determined on each fraction. Total hexoses and galactose were eluted in 6 distinct peaks. The uronic acid containing GAG were eluted in 4 small peaks on G-25 and 1 peak on G-100. This peak is eluted always with high amounts of galactose containing glycoproteins.
