Milder clinical hyperimmunoglobulin E syndrome phenotype is associated with partial interleukin-17 deficiency.
Fulltext:
88899.pdf
Embargo:
until further notice
Size:
335.1Kb
Format:
PDF
Description:
Publisher’s version
Publication year
2010Source
Clinical and Experimental Immunology, 159, 1, (2010), pp. 57-64ISSN
Annotation
01 januari 2010
Publication type
Article / Letter to editor
Display more detailsDisplay less details
Organization
Internal Medicine
Rheumatology
Gastroenterology
Journal title
Clinical and Experimental Immunology
Volume
vol. 159
Issue
iss. 1
Page start
p. 57
Page end
p. 64
Subject
N4i 1: Pathogenesis and modulation of inflammation; N4i 4: Auto-immunity, transplantation and immunotherapy; NCMLS 1: Infection and autoimmunityAbstract
Mutations in the signal transducer and activator of transcription 3 (STAT3) were reported to cause hyperimmunoglobulin E syndrome (HIES). The present study investigates T helper type 17 (Th17) responses triggered by the relevant stimuli Staphylococcus aureus and Candidia albicans in five 'classical' HIES patients, and a family with three patients who all had a milder HIES phenotype. We demonstrate that patients with various forms of HIES have different defects in their Th17 response to S. aureus and C. albicans, and this is in line with the clinical features of the disease. Interestingly, a partial deficiency of interleukin (IL)-17 production, even when associated with STAT3 mutations, leads to a milder clinical phenotype. We also observed defective Th17 responses in patients with the 'classical' presentation of the disease but without STAT3 mutations. These data demonstrate that defective IL-17 production in response to specific pathogens can differ between patients with HIES and that the extent of the defective Th17 response determines their clinical phenotype.
This item appears in the following Collection(s)
- Academic publications [238426]
- Electronic publications [122508]
- Faculty of Medical Sciences [90359]
Upload full text
Use your RU credentials (u/z-number and password) to log in with SURFconext to upload a file for processing by the repository team.