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 molecu-lar level of this defect is being actively examined. In addition, one patient with interferon gamma receptor beta chain deficiency also has been characterized 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. This approach is being investigated for a wide variety of activation associated protein alterations and appears to be useful in screening for patients with severe combined immunodeficiency due to defects in the common gamma chain and JAK3.

Agency
National Institute of Health (NIH)
Institute
Clinical Center (CLC)
Type
Intramural Research (Z01)
Project #
1Z01CL010286-03
Application #
6289481
Study Section
Cognition and Perception Study Section (CP)
Project Start
Project End
Budget Start
Budget End
Support Year
3
Fiscal Year
1999
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