Mucus glycoprotein fatty acyltransferase in patients with cystic fibrosis: effect on the glycoprotein viscosity.
The presence of an acyltransferase activity which catalyzes the transfer of palmitic acid from palmitoyl coenzyme A to mucus glycoprotein has been demonstrated in the microsomal fraction of human rectal mucosa. The activity of this enzyme in the mucosa of patients with cystic fibrosis (CF) was found to be 3.5 times higher than that from normal individuals. The CF mucus glycoprotein in comparison to that of normal contained 1.3 times more associated lipids and 6 times more covalently bound fatty acids. The viscosity of the intact CF glycoprotein was 1.8 times higher than that of normal glycoprotein. Extraction of associated lipids led to 3-fold drop in the viscosity of CF glycoprotein and 5-fold drop in the case of normal glycoprotein. Further loss in the viscosity occurred following removal of the covalently bound fatty acids. The viscosity of such modified CF mucus glycoprotein was only about 10% higher than that of similarly treated normal glycoprotein.
D000102:Acetyl-CoA C-Acyltransferase / Q000378:metabolism*; D000217:Acyltransferases / Q000378:metabolism*; D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002855:Chromatography, Thin Layer; D003550:Cystic Fibrosis / Q000201:enzymology*; D005753:Gastric Mucosa / Q000378:metabolism; D006023:Glycoproteins / Q000032:analysis / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000201:enzymology; D008055:Lipids / Q000032:analysis; D008875:Middle Aged; D009093:Mucus / Q000201:enzymology*; D019308:Palmitic Acid; D010169:Palmitic Acids / Q000378:metabolism; D010171:Palmitoyl Coenzyme A / Q000378:metabolism; D012007:Rectum; D014018:Tissue Distribution; D014783:Viscosity
D006023:Glycoproteins; D008055:Lipids; D010169:Palmitic Acids; D010171:Palmitoyl Coenzyme A; D019308:Palmitic Acid; D000217:Acyltransferases; D000102:Acetyl-CoA C-Acyltransferase