Glycoforms of six serum glycoproteins in a patient with congenital disorder of glycosylation type I (original) (raw)

In this paper the occurrence and relative content of defectively glycosylated serum glycoforms in transferrin (Tf), alpha1-acid glycoprotein (AGP), haptoglobin (Hp), alpha1-antitrypsin (alpha1-AT), alpha2-macroglobulin (alpha2-MG) and ceruloplasmin (Cpl) in the serum of a patient with congenital disorder of glycosylation type I are reported. Blood samples were taken when the patient was 14 years old and then after a one-year interval. The patterns of glycoforms in both samples were compared. In 4 out of 6 examined glycoproteins, glycoforms lacking one and two oligosaccharide chains occurred. "Underglycosylated" glycoforms of alpha2-MG and Cpl were not clearly detectable. Tf was shown to be affected with this defect to a higher extent than other glycoproteins, containing only 30% properly glycosylated molecules and also as much as 30% of the molecules lacking two glycan units. In Hp and alpha1-AT the proportions of properly and defectively glycosylated forms were similar. T...