Human intestinal mucin in cystic fibrosis.
Human intestinal mucins from six subjects with Cystic Fibrosis (CF) and eight subjects without CF were prepared from tissue obtained at surgery (one case) and postmortem. Subjects were not age-matched, but the nonCF mucin was obtained from subjects with ages which bracketed those of the CF subjects. Cesium chloride analytical gradient ultracentrifugation showed that CF mucins were generally denser than nonCF mucins. Sedimentation coefficients were also higher in the CF samples. CF mucins were enriched in fucose, galactose, N-acetylglucosamine and total carbohydrate per mg protein and per oligosaccharide chain (mole/mole GalNAc). Fucose/sialic acid molar ratios were significantly higher in CF mucins, and the average oligosaccharide chain length was approximately three residues greater in CF as compared with nonCF mucins. There was no difference in amino acid profiles or the number of side chains per molecule. The mean sulfate content was higher in the CF mucins but not to a level of significance; however, in the eight mucins, sulfate content correlated positively with total carbohydrate, N-acetylglucosamine and galactose, and therefore increased with oligosaccharide chain length. CF intestinal mucin was therefore denser and more highly glycosylated than nonCF musin and probably contained more sulfate. The increase in glycosylation resulted from a rise in fucose, galactose, and N-acetylglucosamine without a concomitant rise in sialic acid.
D000117:Acetylglucosamine / Q000032:analysis; D000293:Adolescent; D000328:Adult; D000368:Aged; D000596:Amino Acids / Q000032:analysis; D002241:Carbohydrates / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000378:metabolism*; D005260:Female; D005643:Fucose / Q000032:analysis; D005690:Galactose / Q000032:analysis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007421:Intestine, Small / Q000032:analysis*; D008297:Male; D009077:Mucins / Q000032:analysis*; D012794:Sialic Acids / Q000032:analysis
D000596:Amino Acids; D002241:Carbohydrates; D009077:Mucins; D012794:Sialic Acids; D005643:Fucose; D000117:Acetylglucosamine; D005690:Galactose