LYSOSOMAL ACID HYDROLASES IN LIVER IN GARGOYLISM DEFICIENCY OF 4-METHYLUMBELLIFERYL-BETA-GALACTOSIDASE

被引：40

作者：

OCKERMAN, PA

机构：

[1] Department of Clinical Chemistry, University Hospital, Lund

来源：

SCANDINAVIAN JOURNAL OF CLINICAL & LABORATORY INVESTIGATION | 1968年 / 22卷 / 02期

关键词：

Acid hydrolases; Acid phosphatase; Gargoylism; Liver; Lysosomes; αfucosidase; αmannosidase; βacetylglucosaminidase; βgalactosidase; βglucuronidase;

D O I：

10.3109/00365516809160959

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

The activites of six lysosomal acid hydrolases were measured in liver biopsy specimens from six patients with gargoylism and in post-mortem tissue from two patients. A marked diminution was noted for the activity of 4-methylumbelliferyl-βglactosidase in all patients with gargoylism. The deficiency of βgalactosidase did not seem to be due to the presence of an inhibitor in the liver. The activities of βglucuronidase, βacetylglucosaminidase, acid phosphatase and αfucosidase were significantly increased in some of the patients. The activity of αmannosidase was not significantly different from the values in the controls. © 1968 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.

引用

页码：142 / +

页数：1

共 21 条

[1] ABNORMAL SULPHATASE ACTIVITIES IN 2 HUMAN DISEASES ( METACHROMATIC LEUCODYSTROPHY + GARGOYLISM ) [J].