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白细胞中的芳基硫酸酯酶A和B:晚期婴儿型和青少年型异染性脑白质营养不良及对照的比较统计研究

Arylsulfatases A and B in leukocytes: a comparative statistical study of late infantile and juvenile forms of metachromatic leukodystrophy and controls.

作者信息

Dubois G, Turpin J C, Georges M C, Baumann N

出版信息

Biomedicine. 1980 Feb;33(1):2-4.

PMID:6102873
Abstract

We report a statistical study on the level of aryl-sulfatases A and B in leukocytes of 106 controls, 19 cases of metachromatic leukodystrophy (MLD) infantile and juvenile forms and 25 obligate heterozygotes for MLD. Arylsulfatase A has been found to be similarly deficient in patients of the two forms. Half of the mean of the controls have been found in both types for heterozygotes. Arylsulfatase B (ASB) is slightly higher than normal in late infantile MLD although it is not statistically significant. In the 5 cases of the juvenile forms that were examined, ASB was found to be significantly reduced. This enzyme may play a role in relation to the onset of the disease.

摘要

我们报告了一项关于106名对照者、19例婴儿型和青少年型异染性脑白质营养不良(MLD)患者以及25例MLD obligate杂合子白细胞中芳基硫酸酯酶A和B水平的统计研究。已发现两种类型的患者中芳基硫酸酯酶A同样缺乏。杂合子的两种类型中均发现其水平为对照者平均值的一半。芳基硫酸酯酶B(ASB)在晚发型婴儿型MLD中略高于正常水平,尽管无统计学意义。在检查的5例青少年型病例中,发现ASB显著降低。这种酶可能与疾病的发病有关。

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