不同形式的异染性脑白质营养不良（MLD）中芳基硫酸酯酶A的多种分子形式。

Multiple molecular forms of arylsulfatase A in different forms of metachromatic leukodystrophy (MLD).

作者信息

Farrell D F, MacMartin M P, Clark A F

出版信息

Neurology. 1979 Jan;29(1):16-20. doi: 10.1212/wnl.29.1.16.

DOI:10.1212/wnl.29.1.16

PMID:34126

Abstract

Arylsulfatase A (ARA) can be separated into six to eight individual enzymatic bands of activity by isoelectric focusing on cellulose acetate membranes. The residual ARA activity in juvenile metachromatic leukodystrophy (MLD) has a single band of activity with apI of 5.5, whereas the residual ARA in the late infantile form of MLD has three bands of activity with pI range of from 5.4 to 5.8. The technique of isoelectric focusing on cellulose acetate membranes demonstrates enzymatic differences which can be correlated with the clinical form of the disease.

摘要

通过在醋酸纤维素膜上进行等电聚焦，芳基硫酸酯酶A（ARA）可被分离成6至8条具有活性的独立酶带。青少年型异染性脑白质营养不良（MLD）中的残余ARA活性具有一条活性带，其等电点为5.5，而晚期婴儿型MLD中的残余ARA具有三条活性带，其等电点范围为5.4至5.8。在醋酸纤维素膜上进行等电聚焦的技术显示出酶学差异，这些差异可与疾病的临床类型相关联。

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