关于黏多糖贮积症Ⅰ型中肝脏酸性糖胺聚糖的进一步研究。

Further studies on hepatic acidic glycosaminoglycans in the Hurler syndrome.

作者信息

Kofoed J A, Tocci A A, Barceló A C

出版信息

Experientia. 1978 May 15;34(5):577-8. doi: 10.1007/BF01936968.

DOI:10.1007/BF01936968

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.

摘要

从3例黏多糖贮积症I型患者及3名正常对照者的肝脏组织中分离出酸性糖胺聚糖。与正常肝脏相比，黏多糖贮积症I型患者肝脏中的糖醛酸和氨基己糖含量显著升高。黏多糖贮积症I型患者的总糖胺聚糖浓度显著增加（约25倍）。