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红细胞包涵体中β-葡萄糖醛酸酶的出现。

The occurrence of beta-glucuronidase in erythrocyte inclusions.

作者信息

Budde R, Gerok K, von Deimling O, Schaefer H E

机构信息

Pathologisches Institut, Universität Freiburg.

出版信息

Acta Histochem Suppl. 1990;38:195-202.

PMID:1964228
Abstract

This study presents erythrocytic inclusion bodies exhibiting histochemically a strong beta-glucuronidase activity. The unique occurrence of this enzyme in cells of the erythropoietic series has never been described before and characterizes a novel type of erythrocyte inclusions mainly observed in patients suffering from liver damage but also from myelodysplasia and congenital dyserythropoietic anemia type I. Since it is known that hepatocytes contain high activities of beta-glucuronidase, we supposed a relationship between an increased breakdown of liver cells and the appearance of beta-glucuronidase positive inclusions in the red cells. To prove this hypothesis, we determined the beta-glucuronidase plasma levels of 99 unselected patients suffering from various liver disorders in comparison with 19 healthy controls by use of the umbelliferone technique. Our results indicate that in cases with liver diseases beta-glucuronidase plasma levels are significantly increased as compared with normal persons. The availability of a sufficient amount of beta-glucuronidase, thus, seems to be one prerequisite for the appearance of these inclusions in erythrocytes. As demonstrated by our investigations on congenital dyserythropoietic anemia, in which the red cells also show beta-glucuronidase positive inclusions, another premise seems to be an elevated autophagolysosomal activity in the erythrocytes.

摘要

本研究展示了红细胞包涵体,其在组织化学上表现出强烈的β-葡萄糖醛酸酶活性。这种酶在红系细胞中的独特出现此前从未被描述过,它表征了一种新型的红细胞包涵体,主要在肝损伤患者中观察到,但也见于骨髓增生异常综合征和Ⅰ型先天性红细胞生成异常性贫血患者。由于已知肝细胞含有高活性的β-葡萄糖醛酸酶,我们推测肝细胞分解增加与红细胞中β-葡萄糖醛酸酶阳性包涵体的出现之间存在关联。为了验证这一假设,我们使用伞形酮技术,测定了99例未经过挑选的患有各种肝脏疾病的患者以及19名健康对照者的血浆β-葡萄糖醛酸酶水平。我们的结果表明,与正常人相比,患有肝脏疾病的患者血浆β-葡萄糖醛酸酶水平显著升高。因此,有足够量的β-葡萄糖醛酸酶似乎是这些包涵体出现在红细胞中的一个前提条件。正如我们对先天性红细胞生成异常性贫血的研究所表明的,其中红细胞也显示出β-葡萄糖醛酸酶阳性包涵体,另一个前提似乎是红细胞中自噬溶酶体活性升高。

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