The cystic fibrosis ciliary inhibitor (CFCI) has been fractionated from plasma of cystic fibrosis (CF) homozygotes and from the media of cultured fibroblasts derived from CF homozygotes. Plasma and fibroblast media from normal controls have been fractionated in an identical manner. Fractions from plasma and fibroblast culture media that demonstrate ciliary inhibitory activity contain several proteins in a molecular weight range of approximately 5,000-11,000. These proteins have been partially characterized by immunochemical analysis with antisera to 33 human serum proteins. Immunological determinants of albumin, C3 (but not C3a), C4, C5, alpha1-lipoprotein, beta-lipoprotein, beta2-microglobulin and immunoglobulin light chains have been detected by hemagglutination in fractions of CF plasma that inhibited ciliary activity and in analogous fractions from normal sera. None of the proteins were detected in media of cultured fibroblasts from either genotype. Since the same proteins and protein fragments were identified in both CF and normal plasma fractions, and were not detected in CF fibroblast media, it appears that none of these proteins can be identified as the CFCI. Identification of these proteins will permit further purification of the CFCI by immunochemical methods.

1976-01-01·Texas reports on biology and medicine

Effects of cystic fibrosis serum ciliary inhibitor on oyster gill ultrastructure: analysis by scanning and transmission electron microscopy.

Article

作者: Bowman, B H ; Brinkley, B R ; Baur, P S

Ultrastructural analysis including scanning and transmission electron microscopic studies (SEM and TEM) were carried out on oyster gill tissue after exposure either to serum fractions from individuals homozygous or heterozygous for cystic fibrosis (CF), to comparable serum fractions from normal individuals, or to sea water. In 4 of the experiments examined topologically (SEM), the CF sera (either heterozygous or homozygous) stimulated the production of mucus that was found in close association with the cilia. The association of excessive mucus with the cell surface could be responsible, in part, for the well-known inhibition of ciliary activity by a factor in CF serum. In 3 additional SEM experiments involving shorter treatment times, very little difference could be observed between homozygous CF, heterozygous CF, and normal serum-fraction-treated oyster tissues. In parallel experiments, ultrathin sections of gill tissue were examined by means of TEM. Those samples that were responsive, as determined by TEM, displayed several characteristic features, including enlarged and partially exuded goblet cells, altered mucus structure along with twisted and matted cilia. An overall swelling of the gill filament was also observed in the responsive tissues. From TEM analysis no detectable alteration in fine structure was apparent in gill tissues that were treated with sera from heterozygous or normal individuals.

1974-08-01·Life Sciences3区 · 医学

Decreased rate of cytolysis of by cystic fibrosis serum. I. Bioassay and evidence for the possible involvement of a C/F factor — IgC complex

3区 · 医学

Article

作者: Gregory B. Wilson ; Theodore L. Jahn

The ciliotoxic inhibitor associated with cystic fibrosis was assayed on the ciliate protozoan, Colpidium striatum, instead of ciliated tissues.Washed target cells (104 Colpidium) were mixed with different serums or fractions, diluted to a total volume of 1.0 ml with Pringsheim's medium, and observed at 30X magnification at room temperatureAll human serums (normal, homozygous cystic fibrosis, and heterozygote carriers) caused cytolysis of Colpidium; but at a 1:10 dilution, there was a significant decrease in the cytolytic rate induced by 16 out of 19 cystic fibrosis serums, and by 12 out of 16 heterozygote samples.Fractionation and selective absorption indicated that cytolysis was an IgG-controlled mechanism, mediated by complement.Cystic fibrosis serum did not lack complement, and its IgG was bound by Colpidium as well as normal IgG.Since the cystic fibrosis inhibitor was bound to IgG on its constant region, it appeared that the inhibitor and Clq may compete for a similar or neighboring site on the constant region of the IgG mol.This bioassay detected only the inhibitor bound to IgG and was not valuable as a diagnostic tool for cystic fibrosis.

100 项与 Cystic Fibrosis (Prime Medicine) 相关的药物交易

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