Defective Bcl-2 expression in memory B cells from common variable immunodeficiency patients
Common Variable Immunodeficiency
Hypogammaglobulinemia
Naive B cell
DOI:
10.1111/cei.13522
Publication Date:
2020-09-23T01:05:54Z
AUTHORS (6)
ABSTRACT
Common variable immunodeficiency (CVID) is a primary characterized by hypogammaglobulinemia and different degrees of B cell compartment alteration. Memory differentiation requires the orchestrated activation several intracellular signaling pathways that lead to number factors, such as nuclear factor kappa (NF-κB) which, in turn, promote transcriptional programs required for long-term survival. The aim this study was determine if disrupted differentiation, survival cells CVID patients could be related defects pathways. For purpose, we selected readouts reflected strength homeostatic resting cells, protein expression levels Bcl-2 family which transcription promoted NF-κB. We found reduced memory from patients. further explored possible alteration crucial prosurvival pathway analysing mRNAs anti-apoptotic proteins naive mimicking T cell-dependent vitro with CD40L interleukin (IL)-21. BCL-XL mRNA were decreased, together AICDA, after B-cell data suggested molecular mechanism tendency towards apoptosis Lower compromise their survival, less activity NF-κB may condition an inabilityto increase levels, thus not promoting germinal centers.
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