INCREASED EXCRETION OF LOW MOLECULAR WEIGHT GLYCOSAMINOGLYCAN FRAGMENTS FOLLOWING PLASMA INFUSION IN A CASE OF HUNTER SYNDROME

Abstract

A 3-year old boy with the severe form of Hunter syndrome (MPS IIA), was treated by the infusion of plasma from 6 pints of pooled blood over a period of 2 days. Total 24hr uronic acid excretion increased fourfold immediately following infusion. This increase was almost entirely due to glycosaminoglycan fragments not precipitated with 9-aminoacridine although there was also a small increase in the amount of precipitated (polymeric) material. There was an increase in the proportion of glycosaminoglycan fragments of lowest molecular wt. coupled with a decrease in intermediate sized fragments shown by Sephadex gel filtration. Degree of sulphation was stable before infusion, then fell and rose again in a cyclical pattern after treatment. Larged sized precipitable glycosaminoglycan fragments had a noticably higher content of glucosamine than the smaller supernatant fragments suggesting that the heparan sulphate glycosaminoglycans were less readily degradable than dermatan & chondroitin sulphates. This pattern of response differed qualitatively from those we have found in 2 types of (MPS III) (Dean et al, 1973.)

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