内化的α-2-巨球蛋白：α-半乳糖苷酶共轭物在法布里半合子成纤维细胞中的命运

The fate of internalized alpha-2-macroglobulin: alpha-galactosidase conjugate in fibroblasts from Fabry's hemizygote.

作者信息

Osada T, Kuroda Y, Ikai A

出版信息

Biochem Biophys Res Commun. 1987 Mar 30;143(3):954-8. doi: 10.1016/0006-291x(87)90343-3.

DOI:10.1016/0006-291x(87)90343-3

Abstract

In our previous report we described the endocytotic incorporation of coffee bean alpha-galactosidase conjugated to human alpha-2-macroglobulin (alpha-2-M) into cultured fibroblasts derived from a patient with Fabry's disease (1). The fate of internalized alpha-galactosidase according to the method described in the above report is now studied. Measurement of the enzyme activity of subcellular fractions showed that it was concentrated in the lysosomal-mitochondrial fraction. The half-life of internalized alpha-galactosidase was determined to be 2 h.

摘要

在我们之前的报告中，我们描述了与人α-2-巨球蛋白（α-2-M）偶联的咖啡豆α-半乳糖苷酶被内吞进入来自法布里病患者的培养成纤维细胞的过程（1）。现在根据上述报告中描述的方法研究内化的α-半乳糖苷酶的去向。亚细胞组分的酶活性测定表明，它集中在溶酶体-线粒体组分中。内化的α-半乳糖苷酶的半衰期被确定为2小时。

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