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|a pubmed24n0381.xml
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|a (DE-627)NLM114122393
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|a (NLM)11513545
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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|a Bleesing, J J
|e verfasserin
|4 aut
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|a TcR-alpha/beta(+) CD4(-)CD8(-) T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and represents a marker of altered O-glycan biosynthesis
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|c 2001
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|a Text
|b txt
|2 rdacontent
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|a ohne Hilfsmittel zu benutzen
|b n
|2 rdamedia
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|a Band
|b nc
|2 rdacarrier
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|a Date Completed 20.09.2001
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|a Date Revised 08.04.2022
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|a published: Print
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|a Citation Status MEDLINE
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|a Copyright 2001 Academic Press.
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|a Autoimmune lymphoproliferative syndrome (ALPS), caused by inherited defects in apoptosis secondary to mutations in genes encoding Fas/CD95/APO-1 and Fas ligand (Fasl)/CD95L, is characterized by nonmalignant lymphadenopathy and splenomegaly, increased T cell receptor alpha/beta(+) CD4(-)CD8(-) T cells (alpha/beta(+) double-negative T cells [alpha/beta(+)-DNT cells]), autoimmunity, hypergammaglobulinemia, and cytokine abnormalities. The alpha/beta(+)-DNT cells are immunophenotypically and functionally similar to alpha/beta(+)-DNT cells that accumulate in lpr and gld mice, which bear genetic mutations in Fas and FasL. In these mice, alpha/beta(+)-DNT cells express the B-cell-specific CD45R isoform B220. We show that alpha/beta(+)-DNT cells of ALPS patients, with either Fas or FasL mutations, also express B220. In addition, also similar to LPR/gLD mice, they have an unusual population of B220-positive CD4(+) T cells. B220 expression, together with our finding of characteristic lectin binding profiles, demonstrates that cell surface O-linked glycoproteins have undergone specific modifications, which may have consequences for lymphocyte trafficking, cell-cell interactions, and access to alternative apoptosis pathways
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|a Journal Article
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|a Biomarkers
|2 NLM
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|a CD4 Antigens
|2 NLM
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|a CD8 Antigens
|2 NLM
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|a Membrane Glycoproteins
|2 NLM
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|a Polysaccharides
|2 NLM
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|a Protein Isoforms
|2 NLM
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|a Receptors, Antigen, T-Cell, alpha-beta
|2 NLM
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|a Leukocyte Common Antigens
|2 NLM
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|a EC 3.1.3.48
|2 NLM
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1 |
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|a Brown, M R
|e verfasserin
|4 aut
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1 |
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|a Dale, J K
|e verfasserin
|4 aut
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700 |
1 |
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|a Straus, S E
|e verfasserin
|4 aut
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700 |
1 |
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|a Lenardo, M J
|e verfasserin
|4 aut
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700 |
1 |
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|a Puck, J M
|e verfasserin
|4 aut
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1 |
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|a Atkinson, T P
|e verfasserin
|4 aut
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700 |
1 |
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|a Fleisher, T A
|e verfasserin
|4 aut
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773 |
0 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 100(2001), 3 vom: 01. Sept., Seite 314-24
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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773 |
1 |
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|g volume:100
|g year:2001
|g number:3
|g day:01
|g month:09
|g pages:314-24
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|a GBV_USEFLAG_A
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|a SYSFLAG_A
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|a GBV_NLM
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|a GBV_ILN_11
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|a GBV_ILN_24
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|a GBV_ILN_350
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|a AR
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|d 100
|j 2001
|e 3
|b 01
|c 09
|h 314-24
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