Abstract
Utilizing cell-free amniotic fluid in an attempt to establish a technique for the antenatal diagnosis of cystic fibrosis (CF), Bowman et al detected “CF mucociliary inhibitor” in a concentrated fraction of at least one amniotic fluid associated with a homozygous fetus. A number of investigators have attempted to extend observations reported in skin fibroblasts cultivated from patients with CF to cultivated amniotic fluid cells. These include: (1) cellular metachromasia, (2) CF mucociliary inhibitor (CFMI), (3) response to Tamm-Horsfall glycoprotein, (4) enhanced dexamethazone-resistance, and (5) inhibition of oxygen consumption. Significant differences in the extent of protease-methylumbelliferylguanidinobenzoate (MUGB) reactivity were found when plasma and fibroblasts of patients with CF, obligate heterozygotes, and control samples were compared. In these studies, correction for the non-specific hydrolysis of MUGB in crude systems was accomplished by carrying out the reaction in the presence and absence of benzamidine, an efficient competitive inhibitor of trypsin-like enzymes.
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