Marchi A G, Marchi M A, Cigui I, Uxa F, Perticarari S, Nordio S
Helv Paediatr Acta. 1978 Dec;33(6):517-25.
The presence in the serum of both cystic fibrosis (CF) homozygotes and heterozygotes of a factor inhibiting the response of lymphocyte lysosome beta-glucuronidase activity to in vitro phytohaemagglutinin (PHA) stimulation is confirmed. Studying lymphocyte beta-glucuronidase activity on PHA stimulation represents a way to confirm CF diagnosis and to screen CF heterozygotes. For technical complexity, however, the method cannot be used for mass screening, but it can be useful for confirming the diagnosis in suspected cases. Relationships between serum factor inhibiting the effect of PHA on beta-glucuronidase, ciliary dyskinesia factor and carboxypeptidase B-like activity are discussed.
已证实，囊性纤维化（CF）纯合子和杂合子血清中存在一种抑制淋巴细胞溶酶体β-葡萄糖醛酸酶活性对体外植物血凝素（PHA）刺激反应的因子。研究PHA刺激下的淋巴细胞β-葡萄糖醛酸酶活性是确诊CF和筛查CF杂合子的一种方法。然而，由于技术复杂，该方法不能用于大规模筛查，但对疑似病例的诊断确有帮助。文中讨论了抑制PHA对β-葡萄糖醛酸酶作用的血清因子、纤毛运动障碍因子和羧肽酶B样活性之间的关系。
