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The composition of a mucus glycoprotein from meconium of cystic fibrosis, healthy pre-term and full-term neonates.

作者信息

Ryley H C, Rennie D, Bradley D M

出版信息

Clin Chim Acta. 1983 Nov 30;135(1):49-56. doi: 10.1016/0009-8981(83)90387-x.

DOI:10.1016/0009-8981(83)90387-x
PMID:6652918
Abstract

A high molecular mass mucus glycoprotein fraction (molecular mass greater than 1 million) which is a major component of meconium mucin, has been isolated from individual specimens collected from 20 healthy full-term infants, 19 premature infants and 19 infants with proven cystic fibrosis. The mucus glycoprotein fraction isolated from cystic fibrosis meconium had a significantly lower saccharide content than that isolated from specimens from healthy full-term infants but had a similar composition to that isolated from meconium of premature infants, gestational age 28-32 weeks. The composition of the glycoprotein fraction from the meconium of infants, gestational age 32-36 weeks, lay between that from cystic fibrosis and full-term. There is therefore a change or 'maturation' of epithelial mucin during gestation and it is hypothesised that the cystic fibrosis genetic lesion affects the maturation of epithelial secretion, resulting in the abnormal exocrine secretion associated with the disease.

摘要

相似文献

1
The composition of a mucus glycoprotein from meconium of cystic fibrosis, healthy pre-term and full-term neonates.
Clin Chim Acta. 1983 Nov 30;135(1):49-56. doi: 10.1016/0009-8981(83)90387-x.
2
Study of the oligosaccharide units from mucus glycoproteins of meconium from normal infants and from cases of cystic fibrosis with meconium ileus.对正常婴儿胎粪以及患有胎粪性肠梗阻的囊性纤维化病例的胎粪中黏液糖蛋白寡糖单元的研究。
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Abnormal fucosylation of ileal mucus in cystic fibrosis: I. A histochemical study using peroxidase labelled lectins.囊性纤维化患者回肠黏液岩藻糖基化异常:I. 使用过氧化物酶标记凝集素的组织化学研究。
J Clin Pathol. 1990 Dec;43(12):1014-8. doi: 10.1136/jcp.43.12.1014.