Washington University in St. Louis (WU)
I have been an NIH-funded investigator since 1991. My research focuses on the regulation of oxidant production by the phagocyte NADPH oxidase, its role in innate immunity and inflammation and inherited defects in Chronic Granulomatous Disease (CGD), where we recently identified a new genetic subgroup of this disorder. The NADPH oxidase is increasingly recognized to have immunomodulatory functions in addition to its role in microbial killing. My laboratory is investigating the regulation and assembly of NADPH oxidase in phagocytic leukocytes, its role in innate immunity and inflammation, inherited defects in CGD, and strategies for treatment of CGD by gene modification of hematopoietic stem cells. We have developed both a human myeloid cell line model and mouse model of X-linked CGD using gene targeting for use as pre-clinical model systems, in addition to studies in human neutrophils and marrow cells from healthy controls and patients with CGD.