Goldstein J L, Campbell B K, Gartler S M
J Clin Invest. 1972 Apr;51(4):1034-7. doi: 10.1172/JCI106863.
Uncultured human leukocytes contain no detectable cystathionine synthase activity. A method is described in which the addition of phytohemagglutinin (PHA) to short-term lymphocyte cultures results in a significant induction of enzymatic activity. This PHA-stimulated activity has characteristics that resemble those previously described for cystathionine synthase of normal liver and cultured fibroblasts. Lymphocyte cystathionine synthase activity is completely dependent on the presence of homocysteine and is absent or severely deficient in extracts from individuals with the syndrome of homocystinuria. This system for induction of cystathionine synthase in lymphocytes thus provides a simple in vitro technique for (a) diagnosing homocystinuria, (b) studying the mechanism of enzyme regulation and differentiation, and (c) examining the nutritional and hormonal control of cystathionine synthase activity both in normal subjects and homocystinuric patients.
未培养的人白细胞中未检测到胱硫醚合酶活性。本文描述了一种方法,即在短期淋巴细胞培养物中添加植物血凝素(PHA)会导致酶活性显著诱导。这种PHA刺激的活性具有与先前描述的正常肝脏和培养成纤维细胞的胱硫醚合酶相似的特征。淋巴细胞胱硫醚合酶活性完全依赖于同型半胱氨酸的存在,在同型胱氨酸尿症综合征患者的提取物中不存在或严重缺乏。因此,这种在淋巴细胞中诱导胱硫醚合酶的系统为(a)诊断同型胱氨酸尿症、(b)研究酶调节和分化机制以及(c)检查正常受试者和同型胱氨酸尿症患者中胱硫醚合酶活性的营养和激素控制提供了一种简单的体外技术。