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| Description
| - Defekty glykosylace jsou přítomny o mnoha lidských onemocnění. U IgA nefropatie O-glykany přítomné na IgA1 jsou deficientní v galaktóze a jsou tvořeny a2,6 sialylovaným N-acetylgalactosaminem, strkturou suspektní že brání b1,3 galaktosylaci. Tutéž poruchu jsme identifikovali u Dakiki linie B lymfocytů. Biochemické analýzy indikovaly aktivitu CMP-NeuAc:GalNAc-IgA1 a2,6-sialyltransferázy. Nicméně jediný kandidátní gen ST6-GalNAc I nebyl transkribován jak v Dakiki tak v EBV imortalizovaných periferních IgA1 produkujících B lymfocytech od IgAN pacientů a zdravých kontrol. Naopak ST6-GalNAcII transkipt byl průkazný v těchto liniích na vysoké hladině. Exprese byla vyšší v EBV imortalizovaných B lymfocytech z IgAN pacientů ve srovnání se zdravými kontrolami. Z analyzovanýmch krevních vzorků pouze T lymfocyty významně exprimují ST6-GalNAcI. Výzledky ukazují že lidské buňky kterě neexprimují ST6-GalNAcI mohou syalylovat core 1 antigen pomocí ST6-GalNAcII. (cs)
- Glycosylation defects occur in several human diseases.In IgA nephropathy, IgA1 contains O-glycans that are galactose-deficient and consist mostly of core 1 α2,6 sialylated N-acetylgalactosamine, a configuration suspected to prevent β1,3 galactosylation. We confirmed the same aberrancy in IgA1 secreted by the human DAKIKI B cell line. Biochemical assays indicated CMP-NeuAc:GalNAc-IgA1 α2,6-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 α2,6-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
- Glycosylation defects occur in several human diseases.In IgA nephropathy, IgA1 contains O-glycans that are galactose-deficient and consist mostly of core 1 α2,6 sialylated N-acetylgalactosamine, a configuration suspected to prevent β1,3 galactosylation. We confirmed the same aberrancy in IgA1 secreted by the human DAKIKI B cell line. Biochemical assays indicated CMP-NeuAc:GalNAc-IgA1 α2,6-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 α2,6-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)
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| Title
| - Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferase in IgA1-producing Cells
- Identifikace a charakterizace CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferázy v IgA1 produkujících buˇnkách (cs)
- Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferase in IgA1-producing Cells (en)
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| skos:prefLabel
| - Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferase in IgA1-producing Cells
- Identifikace a charakterizace CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferázy v IgA1 produkujících buˇnkách (cs)
- Identification and Characterization of CMP-NeuAc:GalNAc-IgA1 α2,6-Sialyltransferase in IgA1-producing Cells (en)
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| skos:notation
| - RIV/61989592:15110/07:00007190!RIV09-MSM-15110___
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| http://linked.open...avai/riv/aktivita
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| http://linked.open...avai/riv/aktivity
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| http://linked.open...iv/cisloPeriodika
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| http://linked.open...vai/riv/dodaniDat
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| http://linked.open...aciTvurceVysledku
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| http://linked.open.../riv/druhVysledku
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| http://linked.open...iv/duvernostUdaju
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| http://linked.open...titaPredkladatele
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| http://linked.open...dnocenehoVysledku
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| http://linked.open...ai/riv/idVysledku
| - RIV/61989592:15110/07:00007190
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| http://linked.open...riv/jazykVysledku
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| http://linked.open.../riv/klicovaSlova
| - ST6GalNAcII; DAKIKI; IgA nephropathy; IgA1 hinge region; sialylation (en)
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| http://linked.open.../riv/klicoveSlovo
| |
| http://linked.open...odStatuVydavatele
| - GB - Spojené království Velké Británie a Severního Irska
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| http://linked.open...ontrolniKodProRIV
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| http://linked.open...i/riv/nazevZdroje
| - Journal of Molecular Biology
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| http://linked.open...in/vavai/riv/obor
| |
| http://linked.open...ichTvurcuVysledku
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| http://linked.open...cetTvurcuVysledku
| |
| http://linked.open...vavai/riv/projekt
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| http://linked.open...UplatneniVysledku
| |
| http://linked.open...v/svazekPeriodika
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| http://linked.open...iv/tvurceVysledku
| - Julian, Bruce
- Moldoveanu, Zina
- Novák, Jan
- Suzuki, Hitoshi
- Wyatt, Robert
- Raška, Milan
- Tomana, Milan
- Kulhavy, Rose
- Hall, Stacy
- Mestecky, Jiří
- Brown, Rhubell
- Andrasi, Judit
- Carlsson, Fredric
- Lindahl, Gunnar
- Vu, Huong
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| issn
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| number of pages
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| http://localhost/t...ganizacniJednotka
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| is http://linked.open...avai/riv/vysledek
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