Abstract

This grant has for more than a decade provided the core support for a series of studies on patients with primary immunodeficiency syndromes. These patients, as experiments of nature, afford unique opportunities to study specific humoral and cellular immunity as well as non-specific resistance factors in man. Our particular interest has been in defects in antibody formation, assessed by the T-dependent antigen bacteriophage phiX174. It is our hypothesis that many of the syndromes expressed as defects of humoral immunity, particularly the most common defect, common variable immunodeficiency, reflect abnormalities of helper/suppressor cells and associated factors rather than B-cell defects per se. We propose to continue our studies of this problem by monitoring the presence and function of suppressor T-cells by assessing the control mechanisms for immunoglobulin and antibody responses in vivo and in vitro, and by attempting to reconstitute function with modulation of T-cell function by exposure to thymic epithelial monolayers, thymosin and other factors, as well as by transplantation of fetal tissues.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research Project (R01)
Project #
2R01HD017427-21
Application #
3314403
Study Section
Immunological Sciences Study Section (IMS)
Project Start
1978-07-01
Project End
1992-06-30
Budget Start
1987-07-01
Budget End
1988-06-30
Support Year
21
Fiscal Year
1987
Total Cost
Indirect Cost

  Institution

Name
University of Washington
Department
Type
Schools of Medicine
DUNS #
135646524
City
Seattle
State
WA
Country
United States
Zip Code
98195

  Publications

Horiuchi, Katsuyuki; Imai, Kohsuke; Mitsui-Sekinaka, Kanako et al. (2014) Analysis of somatic hypermutations in the IgM switch region in human B cells. J Allergy Clin Immunol 134:411-9
Meyer-Bahlburg, Almut; Renner, Ellen D; Rylaarsdam, Stacey et al. (2012) Heterozygous signal transducer and activator of transcription 3 mutations in hyper-IgE syndrome result in altered B-cell maturation. J Allergy Clin Immunol 129:559-62, 562.e1-2
Albert, Michael H; Notarangelo, Luigi D; Ochs, Hans D (2011) Clinical spectrum, pathophysiology and treatment of the Wiskott-Aldrich syndrome. Curr Opin Hematol 18:42-8
Moratto, Daniele; Giliani, Silvia; Bonfim, Carmem et al. (2011) Long-term outcome and lineage-specific chimerism in 194 patients with Wiskott-Aldrich syndrome treated by hematopoietic cell transplantation in the period 1980-2009: an international collaborative study. Blood 118:1675-84
Schimke, Lena F; Sawalle-Belohradsky, Julie; Roesler, Joachim et al. (2010) Diagnostic approach to the hyper-IgE syndromes: immunologic and clinical key findings to differentiate hyper-IgE syndromes from atopic dermatitis. J Allergy Clin Immunol 126:611-7.e1
Torgerson, Troy R; Genin, Anna; Chen, Chunxia et al. (2009) FOXP3 inhibits activation-induced NFAT2 expression in T cells thereby limiting effector cytokine expression. J Immunol 183:907-15
Renner, Ellen D; Hartl, Dominik; Rylaarsdam, Stacey et al. (2009) Comèl-Netherton syndrome defined as primary immunodeficiency. J Allergy Clin Immunol 124:536-43
Ramesh, Narayanaswamy; Geha, Raif (2009) Recent advances in the biology of WASP and WIP. Immunol Res 44:99-111
Renner, Ellen D; Rylaarsdam, Stacey; Anover-Sombke, Stephanie et al. (2008) Novel signal transducer and activator of transcription 3 (STAT3) mutations, reduced T(H)17 cell numbers, and variably defective STAT3 phosphorylation in hyper-IgE syndrome. J Allergy Clin Immunol 122:181-7
Oxelius, Vivi-Anne; Ochs, Hans D; Hammarstrom, Lennart (2008) Restricted immunoglobulin constant heavy G chain genes in primary immunodeficiencies. Clin Immunol 128:190-8

Showing the most recent 10 out of 95 publications