title: The sugar moiety of Tamm-Horsfall protein is affected by the carbohydrate-deficient glycoprotein type I syndrome. A case study.
abstract: As the sugar moiety of Tamm-Horsfall protein (THP) is affected by many pathological conditions, the aim of this study was to examine the influence of carbohydrate-deficient glycoprotein syndrome (CDG) on THP glycans. THP was isolated from urine of one patient with CDG type I and N-glycan profiling, analysis of monosaccharide content, determination of THP reactivity with specific lectins and with anti-THP antibodies were performed. THP of the CDG patient showed markedly lower amounts of all monosaccharides. Diminished amounts of lactosamine-type chains, galactose and alpha2,3 linked sialic acid were expressed in lower reactivity with PHA-L, DSA and MAA, respectively. These modifications were reflected in altered proportions of tetrasialylated and disialylated oligosaccharide chains. THP of the CDG patient reacted slightly more with anti-THP antibodies. Our results indicate that the CDG type I affects the THP sugar moiety and slightly enhances the THP immunoreactivity.
authors.affiliation: Institute of Biochemistry and Molecular Biology, University of Wroclaw, Poland. grabska@bf.uni.wroc.pl
authors.forename: T
authors.initials: T
authors.lastname: Olczak
authors.forename: M
authors.initials: M
authors.lastname: Olczak
authors.forename: A
authors.initials: A
authors.lastname: Kubicz
mesh_terms: D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D018981:Congenital Disorders of Glycosylation / Q000378:metabolism* / Q000652:urine; D004591:Electrophoresis, Polyacrylamide Gel; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D009005:Monosaccharides / Q000737:chemistry*; D009088:Mucoproteins / Q000737:chemistry* / Q000652:urine; D011134:Polysaccharides / Q000737:chemistry*; D058949:Uromodulin
chemical_list: D009005:Monosaccharides; D009088:Mucoproteins; D011134:Polysaccharides; C548612:UMOD protein, human; D058949:Uromodulin