Heterozygous signal transducer and activator of transcription 3 mutations in hyper-IgE syndrome result in altered B-cell maturation

Despite normal B-cell counts, BAFF-R expression levels were significantly lower in patients with HIES (Fig 2, A). Because plasma BAFF levels can affect BAFF-R expression on B lymphocytes,4 we measured plasma BAFF levels with an ELISA (Quantikine Human BAFF Immunoassay; R&D Systems, Inc, Minneapo...

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Bibliographic Details
Published in:Journal of allergy and clinical immunology 2012-02, Vol.129 (2), p.559-562.e2
Main Authors: Meyer-Bahlburg, Almut, MD, Renner, Ellen D., MD, Rylaarsdam, Stacey, AS, Reichenbach, Janine, MD, Schimke, Lena F., MD, Marks, Amy, DO, Tcheurekdjian, Haig, MD, Hostoffer, Robert, DO, Brahmandam, Archana, MS, Torgerson, Troy R., MD, PhD, Belohradsky, Bernd H., MD, Rawlings, David J., MD, Ochs, Hans D., MD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Aged
Allergy and Immunology
B-Cell Activating Factor - blood
B-Cell Activation Factor Receptor - immunology
B-Lymphocytes - cytology
B-Lymphocytes - immunology
Bacteriophage phi X 174 - immunology
Biological and medical sciences
Case-Control Studies
Cell Growth Processes
Child
Child, Preschool
Female
Fundamental and applied biological sciences. Psychology
Fundamental immunology
Humans
Immune system
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunoglobulins
Immunopathology
Job Syndrome - blood
Job Syndrome - genetics
Job Syndrome - immunology
Male
Medical sciences
Middle Aged
Mutation
Pathogenesis
Plasma
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Signal Transduction
STAT3 Transcription Factor - genetics
STAT3 Transcription Factor - metabolism
Young Adult
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Description
Summary:Despite normal B-cell counts, BAFF-R expression levels were significantly lower in patients with HIES (Fig 2, A). Because plasma BAFF levels can affect BAFF-R expression on B lymphocytes,4 we measured plasma BAFF levels with an ELISA (Quantikine Human BAFF Immunoassay; R&D Systems, Inc, Minneapolis, Minn). Because the number of circulating B cells in patients with STAT3-HIES is normal,7 the observation of increased plasma BAFF levels and parallel reduction in BAFF-R expression was unexpected.
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2011.09.017