Monin B, Gueant J L, Vidailhet M, Michalski J C, Pasquet C, Nicolas J P
Am J Clin Nutr. 1987 May;45(5):981-7. doi: 10.1093/ajcn/45.5.981.
Excretion of haptocorrin (R binder), cobalamin, and other corrinoids was studied in meconium from cystic fibrosis (n = 4), premature (n = 3), and control neonates (n = 13). Corrinoids content was 1.67 +/- 0.92 pmol/mg protein in meconium of cystic fibrosis (CF) neonates but only 0.33 +/- 0.37 and 0.48 +/- 0.47 pmol/mg protein, respectively, in that of prematures and controls. Considering its molecular mass (110,100 +/- 10,100) and its mean isoelectric point (3.67 +/- 0.20), haptocorrin remained undergraded in the meconium of CF neonates whereas it was partially degraded in the meconium of prematures and in most of the meconium from controls. Sequestration of cobalamin by undergraded haptocorrin can explain its increased excretion in CF meconium. Cobalamin-binding capacity of haptocorrin was 22.13 +/- 15.50 pmol/mg protein in CF meconium and about 400-fold lower in meconium of prematures and controls. This may correspond to a fetal intestinal hypersecretion in cases of CF.
对患有囊性纤维化的新生儿(n = 4)、早产儿(n = 3)和对照新生儿(n = 13)的胎粪中运钴胺素(R结合蛋白)、钴胺素及其他类咕啉的排泄情况进行了研究。囊性纤维化(CF)新生儿胎粪中的类咕啉含量为1.67±0.92 pmol/mg蛋白质,而早产儿和对照新生儿胎粪中的类咕啉含量分别仅为0.33±0.37和0.48±0.47 pmol/mg蛋白质。考虑到运钴胺素的分子量(110,100±10,100)及其平均等电点(3.67±0.20),运钴胺素在CF新生儿的胎粪中未被充分降解,而在早产儿的胎粪以及大多数对照新生儿的胎粪中部分降解。未充分降解的运钴胺素对钴胺素的螯合作用可以解释其在CF胎粪中排泄增加的现象。运钴胺素在CF胎粪中的钴胺素结合能力为22.13±15.50 pmol/mg蛋白质,在早产儿和对照新生儿的胎粪中则低约400倍。这可能对应于CF病例中的胎儿肠道分泌亢进。