Neuraminidase assay in cultured human fibroblasts: In situ versus in vitro procedures

被引：2

作者：

Beck, M ^{[1
]}

Scheuring, E ^{[1
]}

Voelter, HU ^{[1
]}

Brandt, J ^{[1
]}

Harzer, K ^{[1
]}

机构：

[1] UNIV TUBINGEN,INST HIRNFORSCH,D-72070 TUBINGEN,GERMANY

来源：

CLINICA CHIMICA ACTA | 1996年 / 251卷 / 02期

关键词：

neuraminidase; sialidosis; mucolipidosis; I-cell disease; galactosialidosis; in situ method;

D O I：

10.1016/0009-8981(96)06304-8

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Further investigations have been carried out to characterize a published procedure of neuraminidase assay, in which the activity is measured directly on the cell culture layer. The pH optimum was 4.0. A V-max value of 130 nmol/mg/h and a K-m of 0.3 mmol/l were found. During incubation in the acid buffer, arylsulphatase activity was released into the medium, whereas neuraminidase activity remained attached to the cells. The in situ method allowed an unequivocal diagnosis of primary and secondary neuraminidase deficiencies. Because of its simplicity and reliability, the method appears useful as a routine method in clinical laboratories.

引用

页码：163 / 171

页数：9

共 19 条

[1] THE ASSAY OF ARYLSULPHATASE-A AND ARYLSULPHATASE-B IN HUMAN URINE [J].