Defined blocks in terminal plasma cell differentiation of common variable immunodeficiency patients
Details
Publication Year 2005-10-15,Volume 175,Issue #8,Page 5498-5503
Journal Title
JOURNAL OF IMMUNOLOGY
Publication Type
Journal Article
Abstract
Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by defective Ab production and recurrent bacterial infections. The largely unknown causes are likely to comprise a diverse set of genetic or acquired defects. In this study, we investigated terminal B cell differentiation in lymph nodes from CVID patients. Up to the germinal center B cell stage, B cell differentiation was normal but terminal plasma cell development. was found to be impaired. Using differential Blimp-1 and Syndecan-1 expression in controls, we defined three different plasma cell subsets that correspond to progressive developmental stages locating to different sites in the lymph node. In the CVID patients, we could only detect one or two of these subsets indicating a defective differentiation. Thus, terminal plasma cell differentiation was found to be impaired despite normal expression of Blimp-1. B cells reaching only the first stage of plasma cell differentiation were further unable to undergo isotype switching and to up-regulate activation markers on B cells stimulated in vitro.
Publisher
AMER ASSOC IMMUNOLOGISTS
Keywords
MEMORY B-CELLS; TRANSCRIPTION FACTOR XBP-1; GREEN FLUORESCENT PROTEIN; GERMINAL-CENTER FORMATION; IMPAIRED UP-REGULATION; HYPER-IGM SYNDROME; BLIMP-1 EXPRESSION; GENE-EXPRESSION; BCL-6; INFLAMMATION
Terms of Use/Rights Notice
Refer to copyright notice on published article.


Creation Date: 2005-10-15 12:00:00
An error has occurred. This application may no longer respond until reloaded. Reload 🗙