BIOCHEMICAL STUDIES ON MUCOPOLYSACCHARIDOSIS Ⅰ. CHARACTERIZATION OF GLYCOSAMINOGLYCANS IN URINE FROM TWO PATIENTS WITH SANFILIPPO'S SYNDROME
- VernacularTitle:粘多糖沉积病的生化研究——Ⅰ、两例Sanfilippo综合征尿氨基多糖的分析
- Author:
Mengyu TIAN
;
Zhenqiang ZHANG
;
- Publication Type:Journal Article
- Keywords:
biochemical analysis;
urine;
glycosaminoglycans;
heparin sulfate;
sanfilippols syndrome
- From:
Journal of Xi'an Jiaotong University(Medical Sciences)
1981;0(03):-
- CountryChina
- Language:Chinese
-
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.
