LEADER 01000caa a22002652 4500
001 NLM228414873
003 DE-627
005 20250215131252.0
007 cr uuu---uuuuu
008 231224s2013 xx |||||o 00| ||eng c
024 7 |a 10.1016/j.clim.2013.05.007  |2 doi 
028 5 2 |a pubmed25n0761.xml 
035 |a (DE-627)NLM228414873 
035 |a (NLM)23773925 
035 |a (PII)S1521-6616(13)00127-7 
040 |a DE-627  |b ger  |c DE-627  |e rakwb 
041 |a eng 
100 1 |a Matharu, Kabir  |e verfasserin  |4 aut 
245 1 0 |a B-cell activating factor (BAFF) is elevated in chronic granulomatous disease 
264 1 |c 2013 
336 |a Text  |b txt  |2 rdacontent 
337 |a ƒaComputermedien  |b c  |2 rdamedia 
338 |a ƒa Online-Ressource  |b cr  |2 rdacarrier 
500 |a Date Completed 17.09.2013 
500 |a Date Revised 03.01.2025 
500 |a published: Print-Electronic 
500 |a Citation Status MEDLINE 
520 |a Published by Elsevier Inc. 
520 |a Chronic Granulomatous Disease (CGD) is an inherited defect in superoxide production leading to life-threatening infections, granulomas, and, possibly, abnormal immunoglobulin concentrations. We investigated whether factors controlling antibody production, such as B-cell activating factor (BAFF), were altered in CGD. CGD subjects had significantly increased mean (2.3-fold, p < 0.0001) plasma concentrations of BAFF compared to healthy donors. Patients on IFN-γ treatment had significantly higher BAFF concentrations compared with CGD patients not taking IFN-γ (1.6-fold, p < 0.005). Leukocytes from CGD subjects produced normal amounts of BAFF in response to IFN-γ or G-CSF in vitro. Expression of BAFF-R and TACI was significantly reduced on CGD B cells. Elevated BAFF in CGD correlated with CRP (R = 0.44), ESR (R = 0.49), and IgM (R = 0.47) and increased rapidly in healthy subjects following intravenous endotoxin administration. These findings suggest that elevated BAFF in CGD subjects and healthy donors is a consequence of acute and chronic inflammation 
650 4 |a Journal Article 
650 4 |a Research Support, N.I.H., Intramural 
650 4 |a Research Support, Non-U.S. Gov't 
650 4 |a B cell activating factor 
650 4 |a B cell receptor 
650 4 |a B-cell activating factor 
650 4 |a BAFF 
650 4 |a BCR 
650 4 |a CGD 
650 4 |a Chronic Granulomatous Disease 
650 4 |a Inflammation 
650 4 |a TACI 
650 4 |a transmembrane activator and CAML-interactor. 
650 7 |a B-Cell Activating Factor  |2 NLM 
650 7 |a Biomarkers  |2 NLM 
650 7 |a Endotoxins  |2 NLM 
650 7 |a Interleukins  |2 NLM 
650 7 |a Tumor Necrosis Factor Ligand Superfamily Member 13  |2 NLM 
650 7 |a Interleukin-21  |2 NLM 
650 7 |a MKM3CA6LT1  |2 NLM 
700 1 |a Zarember, Kol A  |e verfasserin  |4 aut 
700 1 |a Marciano, Beatriz E  |e verfasserin  |4 aut 
700 1 |a Kuhns, Douglas B  |e verfasserin  |4 aut 
700 1 |a Spalding, Christine  |e verfasserin  |4 aut 
700 1 |a Garofalo, Mary  |e verfasserin  |4 aut 
700 1 |a Dimaggio, Thomas  |e verfasserin  |4 aut 
700 1 |a Estwick, Tyra  |e verfasserin  |4 aut 
700 1 |a Huang, Chiung-Yu  |e verfasserin  |4 aut 
700 1 |a Fink, Danielle  |e verfasserin  |4 aut 
700 1 |a Priel, Debra L  |e verfasserin  |4 aut 
700 1 |a Fleisher, Thomas A  |e verfasserin  |4 aut 
700 1 |a Holland, Steven M  |e verfasserin  |4 aut 
700 1 |a Malech, Harry L  |e verfasserin  |4 aut 
700 1 |a Gallin, John I  |e verfasserin  |4 aut 
773 0 8 |i Enthalten in  |t Clinical immunology (Orlando, Fla.)  |d 1999  |g 148(2013), 2 vom: 17. Aug., Seite 258-64  |w (DE-627)NLM098196855  |x 1521-7035  |7 nnns 
773 1 8 |g volume:148  |g year:2013  |g number:2  |g day:17  |g month:08  |g pages:258-64 
856 4 0 |u http://dx.doi.org/10.1016/j.clim.2013.05.007  |3 Volltext 
912 |a GBV_USEFLAG_A 
912 |a SYSFLAG_A 
912 |a GBV_NLM 
912 |a GBV_ILN_11 
912 |a GBV_ILN_24 
912 |a GBV_ILN_350 
951 |a AR 
952 |d 148  |j 2013  |e 2  |b 17  |c 08  |h 258-64