|
|
|
|
| LEADER |
01000naa a22002652 4500 |
| 001 |
NLM118652516 |
| 003 |
DE-627 |
| 005 |
20231222183113.0 |
| 007 |
tu |
| 008 |
231222s2002 xx ||||| 00| ||eng c |
| 028 |
5 |
2 |
|a pubmed24n0396.xml
|
| 035 |
|
|
|a (DE-627)NLM118652516
|
| 035 |
|
|
|a (NLM)11987983
|
| 040 |
|
|
|a DE-627
|b ger
|c DE-627
|e rakwb
|
| 041 |
|
|
|a eng
|
| 100 |
1 |
|
|a Agematsu, Kazunaga
|e verfasserin
|4 aut
|
| 245 |
1 |
0 |
|a Absence of memory B cells in patients with common variable immunodeficiency
|
| 264 |
|
1 |
|c 2002
|
| 336 |
|
|
|a Text
|b txt
|2 rdacontent
|
| 337 |
|
|
|a ohne Hilfsmittel zu benutzen
|b n
|2 rdamedia
|
| 338 |
|
|
|a Band
|b nc
|2 rdacarrier
|
| 500 |
|
|
|a Date Completed 23.05.2002
|
| 500 |
|
|
|a Date Revised 23.02.2022
|
| 500 |
|
|
|a published: Print
|
| 500 |
|
|
|a Citation Status MEDLINE
|
| 520 |
|
|
|a The molecular basis of common variable immunodeficiency (CVID) is unknown. To assess humoral immunity in CVID, we selected 24 patients with early or late onset of disease. X-linked agammaglobulinemia (XLA), X-linked hyper-IgM syndrome (XHIM), and non-XHIM were excluded based on clinical phenotype, assessment of the immune response, presence of Bruton's tyrosine kinase (Btk) in monocytes or platelets, and normal expression of CD40 ligand by activated T cells. The number of circulating B cells was within the normal range or reduced. IgD(-) CD27(+) memory B cells were markedly reduced or absent in all 24 patients and IgD(+) CD27(+) B cells were diminished in 8 patients. Circulating B cells from all 6 patients examined, including CVID patients with IgD(+) CD27(+) cells, failed to undergo somatic hypermutation in immunoglobulin-variable (V)-region genes, similar to cord blood B cells. B cells from CVID patients produced IgM and IgG, but not IgA upon the engagement of Ig receptor and CD40 in the presence of IL-2 and IL-10. B cells from all but 5 patients secreted IgE when stimulated by CD40 crosslinking in the presence of IL-4. The observation of defective memory B cells with abnormal cell marker expression and function demonstrates that naive CVID B cells including those expressing IgD(+) CD27(+), in analogy to cord blood and hyper-IgM syndrome B cells, may be responsible for their failure to differentiate into plasma cells and to produce high-affinity antibodies of different isotypes
|
| 650 |
|
4 |
|a Journal Article
|
| 650 |
|
4 |
|a Research Support, Non-U.S. Gov't
|
| 650 |
|
4 |
|a Research Support, U.S. Gov't, P.H.S.
|
| 650 |
|
7 |
|a Immunoglobulin D
|2 NLM
|
| 650 |
|
7 |
|a Immunoglobulins
|2 NLM
|
| 650 |
|
7 |
|a Tumor Necrosis Factor Receptor Superfamily, Member 7
|2 NLM
|
| 650 |
|
7 |
|a Cytidine Deaminase
|2 NLM
|
| 650 |
|
7 |
|a EC 3.5.4.5
|2 NLM
|
| 700 |
1 |
|
|a Futatani, Takeshi
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Hokibara, Sho
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Kobayashi, Norimoto
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Takamoto, Masaya
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Tsukada, Satoshi
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Suzuki, Harumi
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Koyasu, Shigeo
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Miyawaki, Toshio
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Sugane, Kazuo
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Komiyama, Atsushi
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Ochs, Hans D
|e verfasserin
|4 aut
|
| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 103(2002), 1 vom: 30. Apr., Seite 34-42
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
|
| 773 |
1 |
8 |
|g volume:103
|g year:2002
|g number:1
|g day:30
|g month:04
|g pages:34-42
|
| 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 103
|j 2002
|e 1
|b 30
|c 04
|h 34-42
|