ABSTRACT
Objective To analyze the relationship between soluble brain proteoglycan (PG) and brain development. Methods SD rats were divided into three groups: new-born, young adult and near-old groups. Soluble brain PGs of each group were extracted with 4 mol?L -1 guanidine HCl and purified by DEAE-sephacel chromatography, their molecular sizes were identified by Sepharose 4B chromatography, and their GAG compositions were characterized by two dimensional electrophoresis. Results The extraction amounts of soluble brain proteoglycans were decreased with age; soluble brain PGs could be divided into four fractions according to molecule size. The percentage of the large size CSPG aggregate increased with age, but its HA declined. The percentage of the small sized fraction containing DSPG declined with age increase. Conclusion Soluble brain PGs change with age and are related to brain development.
ABSTRACT
Urine glycosaminoglycans of two patients (full brother and sister )with Sanfilippo's syndrome were analysed by electrophoresis, enzymatic or chemical treatment and ion-exchange chromatography. Heparin sulfate is the main glycosaminoglycan accouting for 70% and 58% of urine glycosaminoglycans in both patients, whereas only 29% and 42% of urine glycosaminoglycans are cnondroitin sulfate. Tbe C/O ratio urine heparin sulfate of the patients is 2.32, indicating that the hexuronic acid component parts of the patient's heparin sulfate are much the same as general heparin sulfate. However, the analysis of glycosaminoglycans with DEAE-Sephadex A-25 chromatography and electrophoresis in 0.1 M HCL showed that urine heparin sulfate of the patients arc heterogeneous, and that it covers, at least, two molecular types with different amount of sulfate and charge density.