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| Content Provider | PubMed Central |
|---|---|
| Author | Ma, Cindy S. Pittaluga, Stefania Avery, Danielle T. Hare, Nathan J. Maric, Irina Klion, Amy D. Nichols, Kim E. Tangye, Stuart G. |
| Copyright Year | 2006 |
| Abstract | Individuals with X-linked lymphoproliferative disease (XLP) display defects in B cell differentiation in vivo. Specifically, XLP patients do not generate a normal number of CD27+ memory B cells, and those few that are present are IgM+. Recent studies have suggested that IgM+CD27+ B cells are not true memory cells, but rather B cells that guard against T cell–independent pathogens. Here we show that human XLP IgM+CD27+ B cells resemble normal memory B cells both morphologically and phenotypically. Additionally, IgM+CD27+ B cells exhibited functional characteristics of normal memory B cells, including the ability to secrete more Ig than naive B cells in response to both T cell–dependent and –independent stimuli. Analysis of spleens from XLP patients revealed a paucity of germinal centers (GCs), and the rare GCs detected were poorly formed. Despite this, Ig variable region genes expressed by XLP IgM+CD27+ B cells had undergone somatic hypermutation to an extent comparable to that of normal memory B cells. These findings reveal a differential requirement for the generation of IgM+ and Ig isotype–switched memory B cells, with the latter only being generated by fully formed GCs. Production of affinity-matured IgM by IgM+CD27+ B cells may protect against pathogens to which a normal immune response is elicited in XLP patients. |
| Related Links | http://dx.doi.org/10.1172/jci25720 |
| Ending Page | 333 |
| Page Count | 12 |
| Starting Page | 322 |
| File Format | |
| ISSN | 00219738 |
| Journal | Journal of Clinical Investigation |
| Issue Number | 2 |
| Volume Number | 116 |
| Language | English |
| Publisher | American Society for Clinical Investigation |
| Publisher Date | 2006-01-01 |
| Access Restriction | Open |
| Rights Holder | American Society for Clinical Investigation |
| Subject Keyword | Medicine(all) Research in Higher Education |
| Content Type | Text |
| Resource Type | Article |
| Subject | Medicine |
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