泰-萨克斯病红细胞基质蛋白的研究
Studies of red cell stromal proteins in Tay-Sachs disease.
作者信息
Balint J A, Kyriakides E C
出版信息
J Clin Invest. 1968 Aug;47(8):1858-64. doi: 10.1172/JCI105876.
Hemoglobin-free red blood cell ghosts of nine patients with Tay-Sachs disease and 14 normal control subjects have been analyzed for content of total protein, hexosamines, individual amino acids, and sialic acid. Red cell ghosts from Tay-Sachs' children have been shown to contain significantly increased amounts of protein, hexosamine, threonine, and serine, and probably sialic acid, each of which was increased by approximately 25% over control values. These observations suggest that the red cell membrane in patients with Tay-Sachs disease contains a significant excess of a glycoprotein or proteins, as compared with normal, and that the metabolic defect in this disease, therefore, affects glycoproteins as well as complex lipids.
对9名患有泰-萨克斯病的患者和14名正常对照者的无血红蛋白红细胞影进行了总蛋白、己糖胺、单个氨基酸和唾液酸含量的分析。结果显示，患有泰-萨克斯病儿童的红细胞影含有显著增加的蛋白质、己糖胺、苏氨酸和丝氨酸，可能还有唾液酸，每种物质的含量均比对照值增加了约25%。这些观察结果表明，与正常人相比，泰-萨克斯病患者的红细胞膜含有显著过量的一种或多种糖蛋白，因此，这种疾病的代谢缺陷不仅影响复合脂质，还影响糖蛋白。
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