Glycoproteins and cystic fibrosis: a review.

This article is a review of the major research of the last 
20 years on abnormal glycoproteins and abnormal glycoprotein metabolism in cystic 
fibrosis. Its purpose is to focus attention on an area considered 
by the author to be of great importance for elucidating the 
primary biochemical defect in CF. The majority of the studies on 
glycoproteins in CF have been done on crude mixtures of glycoproteins 
from tissues or tissue fluids, secretions or excretions. Very few studies 
have compared the properties and composition of purified glycoproteins from CF 
patients and controls. Comparative studies on the enzymes involved in the 
degradation and biosynthesis of the carbohydrate portions of glycoproteins in CF 
patients and normal controls are discussed. Several studies have suggested abnormalities 
in CF alpha-L-fucosidase and CF L-fucose metabolism. There are several studies 
which indirectly suggest that abnormalities in glycoproteins or their metabolism may 
be involved in the pathogenesis of cystic fibrosis. Fruitful areas for 
future investigation are comparison of the properties and composition of specific 
glycoproteins purified to homogeneity from CF and control tissues, particularly with 
regard to carbohydrate composition; comparison of the activities and properties of 
the enzymes involved in both the degradation and biosynthesis of glycoproteins 
in CF and control tissues; and studies of the effects of 
increased electrolyte concentration on the physical-chemical properties of glycoproteins. 