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正常人与戈谢病患者β-葡萄糖脑苷脂酶的免疫学及等电聚焦研究

Immunological and isoelectric focusing study of beta-glucocerebrosidase from normal and Gaucher disease.

作者信息

Hardy B, Hoffman J, Ossimi Z

出版信息

Biochem Biophys Res Commun. 1984 Apr 30;120(2):325-32. doi: 10.1016/0006-291x(84)91257-9.

DOI:10.1016/0006-291x(84)91257-9
PMID:6375656
Abstract

Comparison of normal and Gaucher disease beta-glucocerebrosidase by agarose isoelectric focusing (IEF) demonstrated additional bands at the pI-6 area seen within the mutated enzyme, while both normal placenta and spleen enzyme preparations manifest only major activity at pI-5. Antiglucocerebrosidase antibodies precipitated both normal and pathological enzymes, however, more antibodies were needed to reach an equivalence with the normal enzymes than with the Gaucher's. Cross reactivity of the IEF isozymes were detected by direct immunodiffusion on the prefocused gel.

摘要

通过琼脂糖等电聚焦(IEF)对正常和戈谢病β-葡萄糖脑苷脂酶进行比较,结果显示在突变酶中,在pI - 6区域出现了额外的条带,而正常胎盘和脾脏酶制剂仅在pI - 5处表现出主要活性。抗葡萄糖脑苷脂酶抗体沉淀了正常和病理性酶,然而,与正常酶达到等价所需的抗体比与戈谢病酶所需的更多。通过在预聚焦凝胶上进行直接免疫扩散检测IEF同工酶的交叉反应性。

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Immunological and isoelectric focusing study of beta-glucocerebrosidase from normal and Gaucher disease.正常人与戈谢病患者β-葡萄糖脑苷脂酶的免疫学及等电聚焦研究
Biochem Biophys Res Commun. 1984 Apr 30;120(2):325-32. doi: 10.1016/0006-291x(84)91257-9.
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