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来自患有胡尔勒综合征和谢伊综合征患者的、经爱泼斯坦-巴尔病毒转化的已建立淋巴母细胞样细胞中的α-L-艾杜糖醛酸酶活性。

alpha-L-Iduronidase activity in established lymphoblastoid cells from patients with Hurler and Scheie syndromes transformed by Epstein-Barr virus.

作者信息

Minami R, Suzuki M, Kudoh T, Sato S, Oyanagi K

出版信息

Tohoku J Exp Med. 1977 Aug;122(4):393-6. doi: 10.1620/tjem.122.393.

Abstract

alpha-L-Iduronidase activity was determined in established lymphoblastoid cells, which were transformed in vitro by Epstein-Barr virus, of lymphocytes-rich cell populations isolated from peripheral blood of patients with Hurler and Scheie syndromes. alpha-L-Iduronidase activities in established lymphoblastoid cells from patients were undetectable, while activities of control subjects were clearly detected. These results suggest that established lymphoblastoid cells are useful for the enzymatic study of genetic mucopolysaccharidoses.

摘要

在从患有Hurler综合征和Scheie综合征患者的外周血中分离出的富含淋巴细胞的细胞群体中,对通过爱泼斯坦-巴尔病毒体外转化的已建立的淋巴母细胞样细胞中的α-L-艾杜糖醛酸酶活性进行了测定。患者已建立的淋巴母细胞样细胞中的α-L-艾杜糖醛酸酶活性无法检测到,而对照受试者的活性则可清晰检测到。这些结果表明,已建立的淋巴母细胞样细胞可用于遗传性黏多糖贮积症的酶学研究。

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