Haptoglobin glycoforms in a case of carbohydrate-deficient glycoprotein syndrome |
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Authors: | Mirosława Ferens-Sieczkowska Alina Midro Beata Mierzejewska-Iwanowska Krzysztof Zwierz Iwona Kątnik-Prastowska |
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Affiliation: | (1) Department of Chemistry and Immunochemistry, Wroc aw Medical University, Bujwida 44A, PL-50-345 Wroc aw, Poland;(2) Genetics Division, Institute of Obstetrics and Gynaecology, Poland;(3) Pharmaceutical Biochemistry Division, Institute of Chemistry, Medical Academy, Mickiewicza 2a, PL- 15-222 Bia ystok, Poland |
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Abstract: | Alterations in haptoglobin (Hp) glycosylation were examined in the plasma of the first patient with carbohydrate-deficient glycoprotein syndrome (CDGS) who was described in Poland. Hp concentration in the CDGS patient plasma was low (240mg/l) and the Hp phenotype was shown to be 2-2. Three glycoforms of the Hp subunit were observed in SDS-PAGE in CDGS. The densitometric analysis and molecular weight determinations suggested that 50% of glycoforms were fully glycosylated; 30% contained three out of four and 20% only two out of four glycan units compared to those that are present in Hp derived from healthy people. Results with lectins (concanavalin A and Sambucus nigra, Maackia amurensis and Alleuria aurantia agglutinins) indicate that all three glycoforms of subunit of CDGS-Hp contained biantennary complex glycans terminated with 2,6 bound sialic acid, but without fucose or 2,3 linked sialic acid. Hp glycosylation abnormalities described in this work suggest that this case was a type I carbohydrate-deficient glycoprotein syndrome. |
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Keywords: | carbohydrate-deficient glycoprotein syndrome CDGS, haptoglobin acute phase protein N-glycosylation glycoforms |
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