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X连锁鱼鳞病中角质形成组织的硫酸酯酶活性。

Sulfatase activity of keratinizing tissues in X-linked ichthyosis.

作者信息

Baden H P, Hooker P A, Kubilus J, Tarascio A

出版信息

Pediatr Res. 1980 Dec;14(12):1347-8. doi: 10.1203/00006450-198012000-00015.

Abstract

Arylsulfatases A and B were measured in the stratum corneum of four normal controls and two individuals with sex-linked ichthyosis. For arylsulfatase A, the mean delta optical density/hr/mg protein value was 1.6 for controls and 2.0 for patients, whereas for arylsulfatase B values of 1.5 for controls and 1.4 for patients were observed. Assay of arylsulfatase C in the callus of four normal controls showed a mean delta optical density/hr/100 mg callus of 0.63, whereas no or trace activity was detected in callus from four patients with x-linked ichthyosis. The assay of steroid sulfatase is best for studying microsomal sulfatase activity. Table 1 shows the activity of this enzyme in nails, callus, and hair bulbs from controls and patients with x-linked ichthyosis. No steroid sulfatase could be demonstrated in patients with x-linked ichthyosis. The values in normal controls and obligate heterozygotes are compared in Table 2. The mean value of the two groups is statistically different with P less than or equal to 0.05 using the Student t test.

摘要

在四名正常对照者和两名性联鱼鳞病患者的角质层中检测了芳基硫酸酯酶A和B。对于芳基硫酸酯酶A,对照者的平均光密度变化/小时/毫克蛋白值为1.6,患者为2.0;而对于芳基硫酸酯酶B,对照者的值为1.5,患者为1.4。对四名正常对照者的老茧进行芳基硫酸酯酶C检测,结果显示平均光密度变化/小时/100毫克老茧为0.63,而在四名X连锁鱼鳞病患者的老茧中未检测到或仅检测到微量活性。类固醇硫酸酯酶检测最适合用于研究微粒体硫酸酯酶活性。表1显示了该酶在正常对照者和X连锁鱼鳞病患者的指甲、老茧和毛球中的活性。在X连锁鱼鳞病患者中未检测到类固醇硫酸酯酶。表2比较了正常对照者和肯定杂合子的值。使用学生t检验,两组的平均值在统计学上有差异,P小于或等于0.05。

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