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Journal of Clinical Immunology and Allergy
ISSN: 2471-304X
16
th
EuroSciCon Conference on
Immunology
M a r c h 1 1 - 1 2 , 2 0 1 9
Am s t e r d a m , N e t h e r l a n d s
Immunology 2019
X
-linked Agammaglobulinemia (XLA) is a prototype primary antibody deficiency which is the most common form of primary
immunodeficiency diseases. Mutated BTK in these patients affect many immune cells, immunologic responses and molecular
interactions. TLRs, in a close interaction with BTK, reported being defected in different subcellular populations of PBMCs of XLA
patients. In this concern, we aimed to assess LPS and CpG-A stimulatory action on TLR4 and TLR9 by measuring the activation
of some TLR negative regulatory molecules’ transcription and cytokine production. Higher transcripts of SOCS1 and RNF216
were found in unstimulated PBMCs of patients. Despite this, interesting patterns of TLR-induced transcription were observed:
upregulation of IRAKM and SOCS1 in healthy subjects but downregulation in XLA, lack of RNF216 induction in healthy subjects
while downregulation in patients and similar TNFAIP3 downregulation in both XLA and healthy subjects. Further, a lower amount
of TNF-α was also produced by XLA patients PBMCs after LPS stimulation by disturbed cytokine production and dysregulated
transcription of selected downstream signalling molecules. Our results strengthen the potential TLRs defect pointing out TLR
involvement in the pathogenesis of different complications of XLA patients and also the scale of this defectiveness form TLRs
expression to downstream signalling and cytokine production.
rsanaei.rs@gmail.comDisturbed gene expression of TLR negative
regulators in XLA patients
Roozbeh Sanaei
1
, Nima Rezaei
2
, Asghar Aghamohammadi
3
and Nader Tajik
1
1
Immunology Research Center (IRC), Institute of Immunology and Infectious Diseases, Iran
2
NIIMA, Universal Scientific Education and Research Network (USERN), Iran
3
Tehran University of Medical Sciences, Iran
J Clin Immunol Allergy 2019, Volume:5
DOI: 10.21767/2471-304X-C1-009