title: Toll-like receptor stimulation induces higher TNF-alpha secretion in peripheral blood mononuclear cells from patients with hyper IgE syndrome.
abstract: 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.
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Medical Sciences/University of Tehran, Tehran, Iran.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Yeganeh
authors.forename: Philipp
authors.initials: P
authors.lastname: Henneke
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Stephan
authors.initials: S
authors.lastname: Ehl
authors.forename: Doerte
authors.initials: D
authors.lastname: Thiel
authors.forename: Nuria
authors.initials: N
authors.lastname: Matamoros
authors.forename: Cristina
authors.initials: C
authors.lastname: Pietrogrande
authors.forename: Teresa
authors.initials: T
authors.lastname: Espanol
authors.forename: Jiri
authors.initials: J
authors.lastname: Litzman
authors.forename: Jose L
authors.initials: JL
authors.lastname: Franco
authors.forename: Ozden
authors.initials: O
authors.lastname: Sanal
authors.forename: Sara S
authors.initials: SS
authors.lastname: Kilic
authors.forename: Anna
authors.initials: A
authors.lastname: Breborowicz
authors.forename: Alessandro
authors.initials: A
authors.lastname: Plebani
authors.forename: Ellen
authors.initials: E
authors.lastname: Renner
authors.forename: Simon
authors.initials: S
authors.lastname: Rothenfusser
authors.forename: Thomas R
authors.initials: TR
authors.lastname: Hawn
authors.forename: Cristina
authors.initials: C
authors.lastname: Woellner
authors.forename: Bodo
authors.initials: B
authors.lastname: Grimbacher
mesh_terms: 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*
chemical_list: 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