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.

• Autores: Franco Restrepo, José Luis; Yeganeh, Mehdi; Henneke, Philipp; Rezaei, Nima; Ehl, Stephan; Thiel, Doerte; Matamoros, Nuria; Pietrogrande, Cristina; Espanol, Teresa; Litzman, Jiri; Sanal, Ozden; Kilic, Sara S.; Breborowicz, Anna; Plebani, Alessandro; Renner, Ellen; Rothenfusser, Simon; Hawn, Thomas R.; Woellner, Cristina; Grimbacher, Bodo
• Tipo de recurso: Article of investigation
• Fecha de publicación: 2008
• Institución: Universidad de Antioquia
• Repositorio: Repositorio UdeA
• Idioma: eng
• OAI Identifier: oai:bibliotecadigital.udea.edu.co:10495/43125
• Acceso en línea: https://hdl.handle.net/10495/43125
• Palabra clave: Cohort Studies; Estudios de Cohortes; Interleukin-8; Interleucina-8; Job Syndrome; Síndrome de Job; Leukocytes, Mononuclear; Leucocitos Mononucleares; Lipopolysaccharides; Lipopolisacáridos; Signal Transduction; Transducción de Señal; Statistics, Nonparametric; Estadísticas no Paramétricas; Toll-Like Receptor 2; Toll-Like Receptor 4; Receptor Toll-Like 4; Receptor Toll-Like 2; Tumor Necrosis Factor-alpha; Factor de Necrosis Tumoral alfa; https://id.nlm.nih.gov/mesh/D015331; https://id.nlm.nih.gov/mesh/D016209; https://id.nlm.nih.gov/mesh/D007589; https://id.nlm.nih.gov/mesh/D007963; https://id.nlm.nih.gov/mesh/D008070; https://id.nlm.nih.gov/mesh/D015398; https://id.nlm.nih.gov/mesh/D018709; https://id.nlm.nih.gov/mesh/D051195; https://id.nlm.nih.gov/mesh/D051197; https://id.nlm.nih.gov/mesh/D014409
• Rights: openAccess
• License: https://creativecommons.org/licenses/by-nc/4.0/