囊性纤维化胎粪的研究：α-D-甘露糖苷酶、β-葡萄糖醛酸酶、β-D-岩藻糖苷酶、酸性和碱性磷酸酶的活性

Studies in meconium in cystic fibrosis: the activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase.

作者信息

Antonowicz I, Ishida S, Shwachman H

出版信息

Biol Neonate. 1978;34(5-6):225-30. doi: 10.1159/000241133.

Abstract

The specific activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase were studied in meconium from infants with cystic fibrosis (CF) and control subjects. The study revealed significant variations in the specific activity of the enzymes except for acid phosphatase. The variations were not uniform. The activities of alpha-D-mannosidase, beta-glucuronidase and alkaline phosphatase were markedly decreased (p less than 0.001, p less than 0.002, p less than 0.001, respectively), while the activity of beta-D-fucosidase was significantly increased (p less than 0.001) in meconium from the infants with CF. It is suggested that the decreased activity of alpha-D-mannosidase and beta-glucuronidase might contribute to the accumulation of the abnormal substances in CF meconium. The highly increased activity of beta-D-fucosidase raises the possibility of an additional or alternative method for screening newborns for CF using meconium as the test material.

摘要

对患有囊性纤维化（CF）的婴儿和对照受试者的胎粪中α-D-甘露糖苷酶、β-葡萄糖醛酸酶、β-D-岩藻糖苷酶、酸性和碱性磷酸酶的比活性进行了研究。研究发现，除酸性磷酸酶外，这些酶的比活性存在显著差异。这些差异并不一致。CF婴儿胎粪中α-D-甘露糖苷酶、β-葡萄糖醛酸酶和碱性磷酸酶的活性显著降低（分别为p<0.001、p<0.002、p<0.001），而β-D-岩藻糖苷酶的活性显著升高（p<0.001）。有人认为，α-D-甘露糖苷酶和β-葡萄糖醛酸酶活性的降低可能导致CF胎粪中异常物质的积累。β-D-岩藻糖苷酶的高度升高增加了以胎粪为检测材料对新生儿进行CF筛查的另一种或替代方法的可能性。