黏多糖贮积症Ⅰ型：对培养的淋巴细胞系的一项研究。
The Hurler syndrome: a study of cultured lymphoid cell lines.
作者信息
Danes B S, Hütteroth T H, Cleve H, Bearn A G
出版信息
J Exp Med. 1972 Sep 1;136(3):644-9. doi: 10.1084/jem.136.3.644.
Abstract
Lymphoid suspension lines have been established from three patients with the Hurler syndrome and four normals. The Hurler lines can be distinguished from normals by (a) staining characteristics, (b) increase in total cellular mucopolysaccharide content, and (c) increase in dermatan sulfate. Hyaluronic acid is absent in cultured lymphoid cells from normal persons and patients with the Hurler syndrome. The availability of biochemically marked suspension cultures should prove useful for enzymatic studies as well as for further elucidation of this clinical syndrome.
摘要
已从三名患有胡尔勒综合征的患者和四名正常人中建立了淋巴悬浮细胞系。胡尔勒细胞系可通过以下方面与正常细胞系区分开来：（a）染色特性，（b）总细胞粘多糖含量增加，以及（c）硫酸皮肤素增加。正常人和胡尔勒综合征患者的培养淋巴细胞中均不存在透明质酸。具有生化标记的悬浮培养物的可用性对于酶学研究以及进一步阐明这种临床综合征应会证明是有用的。
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