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The effects of RelB deficiency on lymphocyte development and function.
Nigel Sharfe1, Daniele Merico2, Ariana Karanxha1
1The Canadian Centre for Primary Immunodeficiency, Immunogenomic Laboratory, Division of Immunology/Allergy, Department of Pediatrics, The Hospital for Sick Children and the University of Toronto, 555 University Ave., Toronto, On M5G 1X8, Canada.
Journal of Autoimmunity
|September 20, 2015
Summary
RelB deficiency impairs human immune cell development, leading to combined immunodeficiency and autoimmunity. This study reveals RelB
Area of Science:
- Immunology
- Molecular Biology
- Genetics
Background:
- The NFκB pathway regulates cell growth and inflammation, with two known pathways.
- The alternative NFκB pathway, mediated by RelB, has an unclear role in human immunity.
- Mutations in RelB are linked to combined immunodeficiency and autoimmunity.
Observation:
- RelB deficiency causes thymic dysplasia and reduced thymus emigrants.
- Accumulation of CD45 RO(+) T cells and CD62L(+) central memory cells observed.
- Skewed T cell receptor repertoire with clonal expansion noted.
Findings:
- Reduced in vitro responses to T cell mitogens and decreased IL2/IFNγ production.
- Impaired TH1 differentiation with reduced T bet and STAT1.
- Aberrant B cell development, arrested B cell maturation, and absent CD27(+) memory cells.
- Reduced BAFF-R and aberrant CD40 signaling.
Implications:
- RelB is crucial for human lymphocyte development, impacting both T and B cell maturation.
- RelB deficiency leads to impaired antibody production and immune responses.
- Skewed T cell repertoire may drive autoimmune features observed in patients.
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