. "Ra\u0161ka, Milan" . "369" . "Wyatt, Robert" . "15"^^ . . "425278" . "Lindahl, Gunnar" . "Julian, Bruce" . "Glycosylation defects occur in several human diseases.In IgA nephropathy, IgA1 contains O-glycans that are galactose-deficient and consist mostly of core 1 \u03B12,6 sialylated N-acetylgalactosamine, a configuration suspected to prevent \u03B21,3 galactosylation. We confirmed the same aberrancy in IgA1 secreted by the human DAKIKI B cell line. Biochemical assays indicated CMP-NeuAc:GalNAc-IgA1 \u03B12,6-sialyltransferase activity in this cell line. However, a candidate enzyme, ST6-GalNAcI, was not transcribed in DAKIKI cells, B cells isolated from blood, or Epstein-Barr virus (EBV)-immortalized IgA1-producing cells from the blood of IgAN patients and healthy controls. Instead, ST6-GalNAcII transcription was detected at a high level. Expression of the ST6-GalNAcII gene and activity of the CMP-NeuAc:GalNAc-IgA1 \u03B12,6-sialyltransferase were higher in IgA1-producing cell lines from IgAN patients than in such cells from healthy controls. These data are the first evidence that human cells that lack ST6" . "10"^^ . "ST6GalNAcII; DAKIKI; IgA nephropathy; IgA1 hinge region; sialylation"@en . "15110" . "0022-2836" . "Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransferase in IgA1-producing Cells"@en . "P(ME 875)" . "1" . "Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransferase in IgA1-producing Cells"@en . . "Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransferase in IgA1-producing Cells" . "[A8BA6421439D]" . "Nov\u00E1k, Jan" . . . "Journal of Molecular Biology" . "Andrasi, Judit" . . "RIV/61989592:15110/07:00007190" . "Kulhavy, Rose" . "Identifikace a charakterizace CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransfer\u00E1zy v IgA1 produkuj\u00EDc\u00EDch bu\u02C7nk\u00E1ch"@cs . "RIV/61989592:15110/07:00007190!RIV09-MSM-15110___" . . "GB - Spojen\u00E9 kr\u00E1lovstv\u00ED Velk\u00E9 Brit\u00E1nie a Severn\u00EDho Irska" . "Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransferase in IgA1-producing Cells" . "Suzuki, Hitoshi" . . . "Mestecky, Ji\u0159\u00ED" . "Glycosylation defects occur in several human diseases.In IgA nephropathy, IgA1 contains O-glycans that are galactose-deficient and consist mostly of core 1 \u03B12,6 sialylated N-acetylgalactosamine, a configuration suspected to prevent \u03B21,3 galactosylation. We confirmed the same aberrancy in IgA1 secreted by the human DAKIKI B cell line. Biochemical assays indicated CMP-NeuAc:GalNAc-IgA1 \u03B12,6-sialyltransferase activity in this cell line. However, a candidate enzyme, ST6-GalNAcI, was not transcribed in DAKIKI cells, B cells isolated from blood, or Epstein-Barr virus (EBV)-immortalized IgA1-producing cells from the blood of IgAN patients and healthy controls. Instead, ST6-GalNAcII transcription was detected at a high level. Expression of the ST6-GalNAcII gene and activity of the CMP-NeuAc:GalNAc-IgA1 \u03B12,6-sialyltransferase were higher in IgA1-producing cell lines from IgAN patients than in such cells from healthy controls. These data are the first evidence that human cells that lack ST6"@en . "1"^^ . . "Identifikace a charakterizace CMP-NeuAc:GalNAc-IgA1 \u03B12,6-Sialyltransfer\u00E1zy v IgA1 produkuj\u00EDc\u00EDch bu\u02C7nk\u00E1ch"@cs . . . "Hall, Stacy" . . "Defekty glykosylace jsou p\u0159\u00EDtomny o mnoha lidsk\u00FDch onemocn\u011Bn\u00ED. U IgA nefropatie O-glykany p\u0159\u00EDtomn\u00E9 na IgA1 jsou deficientn\u00ED v galakt\u00F3ze a jsou tvo\u0159eny a2,6 sialylovan\u00FDm N-acetylgalactosaminem, strkturou suspektn\u00ED \u017Ee br\u00E1n\u00ED b1,3 galaktosylaci. Tut\u00E9\u017E poruchu jsme identifikovali u Dakiki linie B lymfocyt\u016F. Biochemick\u00E9 anal\u00FDzy indikovaly aktivitu CMP-NeuAc:GalNAc-IgA1 a2,6-sialyltransfer\u00E1zy. Nicm\u00E9n\u011B jedin\u00FD kandid\u00E1tn\u00ED gen ST6-GalNAc I nebyl transkribov\u00E1n jak v Dakiki tak v EBV imortalizovan\u00FDch perifern\u00EDch IgA1 produkuj\u00EDc\u00EDch B lymfocytech od IgAN pacient\u016F a zdrav\u00FDch kontrol. Naopak ST6-GalNAcII transkipt byl pr\u016Fkazn\u00FD v t\u011Bchto lini\u00EDch na vysok\u00E9 hladin\u011B. Exprese byla vy\u0161\u0161\u00ED v EBV imortalizovan\u00FDch B lymfocytech z IgAN pacient\u016F ve srovn\u00E1n\u00ED se zdrav\u00FDmi kontrolami. Z analyzovan\u00FDmch krevn\u00EDch vzork\u016F pouze T lymfocyty v\u00FDznamn\u011B exprimuj\u00ED ST6-GalNAcI. V\u00FDzledky ukazuj\u00ED \u017Ee lidsk\u00E9 bu\u0148ky kter\u011B neexprimuj\u00ED ST6-GalNAcI mohou syalylovat core 1 antigen pomoc\u00ED ST6-GalNAcII."@cs . "Brown, Rhubell" . "Moldoveanu, Zina" . "Tomana, Milan" . "Vu, Huong" . . . . . "Carlsson, Fredric" .