Toll-like receptor stimulation induces higher TNF-alpha secretion in peripheral blood mononuclear cells from patients with hyper IgE syndrome.
Hyper IgE syndromes (HIES) are primary immunodeficiency disorders of unknown pathogenesis. Patients are typically affected with 'cold' abscesses of the skin, recurrent cyst-forming pneumonia, chronic mucocutaneous candidiasis and other less frequent features such as progressive skeletal abnormalities. Defective signaling in the Toll-like receptor (TLR) pathways has been suggested as a responsible pathologic mechanism, however, in previous reports, 10 patients revealed no defect in inflammatory cytokine responses to different TLR ligands. Here, we report the increase in pro-inflammatory cytokines TNF-alpha and IL-8, following TLR2 and TLR4 stimulation in a larger cohort of 25 additional patients with HIES, and provide a meta-analysis of the TLR data in HIES.
Yeganeh
Mehdi
M
Immunology, Asthma and Allergy Research Institute, Medical Sciences/University of Tehran, Tehran, Iran.
Henneke
Philipp
P
Rezaei
Nima
N
Ehl
Stephan
S
Thiel
Doerte
D
Matamoros
Nuria
N
Pietrogrande
Cristina
C
Espanol
Teresa
T
Litzman
Jiri
J
Franco
Jose L
JL
Sanal
Ozden
O
Kilic
Sara S
SS
Breborowicz
Anna
A
Plebani
Alessandro
A
Renner
Ellen
E
Rothenfusser
Simon
S
Hawn
Thomas R
TR
Woellner
Cristina
C
Grimbacher
Bodo
B
D015331:Cohort Studies; D005260:Female; D006801:Humans; D016209:Interleukin-8 / Q000097:blood / Q000276:immunology*; D007589:Job Syndrome / Q000276:immunology*; D007963:Leukocytes, Mononuclear / Q000276:immunology*; D008070:Lipopolysaccharides / Q000276:immunology; D008297:Male; D015398:Signal Transduction; D018709:Statistics, Nonparametric; D051195:Toll-Like Receptor 2 / Q000276:immunology*; D051197:Toll-Like Receptor 4 / Q000276:immunology*; D014409:Tumor Necrosis Factor-alpha / Q000097:blood / Q000276:immunology*
D016209:Interleukin-8; D008070:Lipopolysaccharides; C495342:TLR2 protein, human; C495345:TLR4 protein, human; D051195:Toll-Like Receptor 2; D051197:Toll-Like Receptor 4; D014409:Tumor Necrosis Factor-alpha