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使用4-甲基伞形酮基-α-L-艾杜糖苷酸对α-L-艾杜糖醛酸酶活性进行荧光测定。

Fluorometric measurement of alpha-L-iduronidase activity using 4-methylumbelliferyl-alpha-L-iduronide.

作者信息

Minami R, Watanabe Y, Kudoh T, Oyanagi K, Nakao T

出版信息

Tohoku J Exp Med. 1980 Apr;130(4):381-4. doi: 10.1620/tjem.130.381.

Abstract

Using 4-methylumbelliferyl-alpha-L-iduronide as a substrate, alpha-L-iduronidase activity was measured in leukocytes and in lymphoblastoid cells obtained from patients with alpha-L-iduronidase deficiency and from obligate heterozygotes for this disease. There was complete discrimination between alpha-L-iduronide in leukocytes and in lymphoblastoid cells from the patients and controls. However, overlap was observed between values of the activity in the obligate heterozygotes and those in the controls. 4-Methylumbelliferyl-alpha-L-iduronidase activity because of greater sensitivity, easier assay procedure and shorter incubation period.

摘要

以 4-甲基伞形酮基-α-L-艾杜糖醛酸苷作为底物,在从α-L-艾杜糖醛酸酶缺乏症患者以及该病的 obligate 杂合子中获取的白细胞和淋巴母细胞样细胞中测量α-L-艾杜糖醛酸酶活性。患者和对照的白细胞及淋巴母细胞样细胞中的α-L-艾杜糖醛酸之间存在完全的区分。然而,在 obligate 杂合子的活性值与对照的活性值之间观察到有重叠。4-甲基伞形酮基-α-L-艾杜糖醛酸酶活性因其更高的灵敏度、更简便的测定程序和更短的温育时间。

“obligate heterozygotes”可译为“必然杂合子”,但此表述在医学领域可能不太常见,具体意思可结合更专业的医学知识理解,这里暂按原样保留英文以便准确传达原文信息。 最后一句表述不太完整准确,推测完整可能是在阐述选择该物质作为底物来检测酶活性的原因等,但仅根据现有文本只能这样翻译。

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