Document Detail


Haptoglobin glycoforms in a case of carbohydrate-deficient glycoprotein syndrome.
MedLine Citation:
PMID:  10972135     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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 (240 mg/l) and the Hp phenotype was shown to be 2-2. Three glycoforms of the Hp beta 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 beta subunit of CDGS-Hp contained biantennary complex glycans terminated with alpha2,6 bound sialic acid, but without fucose or alpha2,3 linked sialic acid. Hp glycosylation abnormalities described in this work suggest that this case was a type I carbohydrate-deficient glycoprotein syndrome.
Authors:
M Ferens-Sieczkowska; A Midro; B Mierzejewska-Iwanowska; K Zwierz; I Katnik-Prastowska
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Glycoconjugate journal     Volume:  16     ISSN:  0282-0080     ISO Abbreviation:  Glycoconj. J.     Publication Date:  1999 Oct 
Date Detail:
Created Date:  2000-09-25     Completed Date:  2000-09-25     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8603310     Medline TA:  Glycoconj J     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  573-7     Citation Subset:  IM    
Affiliation:
Department of Chemistry and Immunochemistry, Wrocław Medical University, Poland.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Amidohydrolases / metabolism
Blotting, Western
Carbohydrate-Deficient Glycoprotein Syndrome / blood*,  classification,  physiopathology
Child
Electrophoresis, Polyacrylamide Gel
Female
Fucose / analysis
Glycosylation
Haptoglobins / chemistry*,  metabolism
Humans
Infant
Lectins / metabolism
Molecular Weight
N-Acetylneuraminic Acid / analysis
Neuraminidase / metabolism
Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase
Poland
Polysaccharides / analysis,  chemistry,  metabolism
Protein Binding
Protein Isoforms / blood,  chemistry,  metabolism
Chemical
Reg. No./Substance:
0/Haptoglobins; 0/Lectins; 0/Polysaccharides; 0/Protein Isoforms; 131-48-6/N-Acetylneuraminic Acid; 3713-31-3/Fucose; EC 3.2.1.18/Neuraminidase; EC 3.5.-/Amidohydrolases; EC 3.5.1.52/Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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