INCREASED HEPARIN BINDING IN CYSTIC FIBROSIS: A REFLECTION OF ALTERED GLYCOPROTEIN BIOSYNTHESIS?“

Abstract

Difference between the ability of serum proteins to bind with heparin (heparin binding capacity) in children with cystic fibrosis and in normal individuals was studied in an in vitro system. Results indicate the ‘heparin binding capacity’ of serum proteins in individuals with cystic fibrosis is significantly increased compared with normal subjects and quantitatively increases with progressing severity of the disease. By weight the combining ratio of protein to heparin is twenty-five per cent (25%) lower in cystic fibrosis affected individuals and is independent of the severity of the disease process. Some of the proteins which bind to heparin were characterized as glycoproteins; those from cystic fibrosis serum were twenty-seven per cent (27%) higher in fucose (methylpentose) content, twenty-seven per cent (27%) lower in sialic acid content, and thirty-one per cent (31%) lower in hexose content when compared to heparin precipitated serum glycoproteins from control subjects. Hexosamine content of the heparin precipitated serum glycoproteins was not altered. The results of the investigation suggest that glycoprotein biosynthesis is altered in cystic fibrosis.

Spon. by grant #81-779, Children's Hospital Research Foundation.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD