Monocytes are being characterized for the expression levels of CD40, CD80, CD86, CD120b, and the gamma interferon receptor alpha chain. These studies have identified two patients with gamma interferon receptor alpha chain deficiency, and the molecular level of this defect is being actively examined. In addition, interferon gamma receptor beta chain deficiency has also been characterized in one patient at the molecular level. As a consequence of these studies, an intracellular flow cytometric method for evaluating STAT protein phosphorylation has been developed and validated initially for STAT1 and more recently for STAT4. The combination of cell surface receptor and functional status at the level of cytokine response is being applied to new patients and is also being used to evaluate patients after bone marrow transplantation.

Agency
National Institute of Health (NIH)
Institute
Clinical Center (CLC)
Type
Intramural Research (Z01)
Project #
1Z01CL010286-05
Application #
6542081
Study Section
Cognition and Perception Study Section (CP)
Project Start
Project End
Budget Start
Budget End
Support Year
5
Fiscal Year
2001
Total Cost
Indirect Cost
Name
Clinical Center
Department
Type
DUNS #
City
State
Country
United States
Zip Code
Bleesing, J J; Fleisher, T A (2001) Cell function-based flow cytometry. Semin Hematol 38:169-78
Uzel, G; Frucht, D M; Fleisher, T A et al. (2001) Detection of intracellular phosphorylated STAT-4 by flow cytometry. Clin Immunol 100:270-6
Fleisher, T A; Dorman, S E; Anderson, J A et al. (1999) Detection of intracellular phosphorylated STAT-1 by flow cytometry. Clin Immunol 90:425-30