Chronic granulomatous disease.
Chronic granulomatous disease is an inherited disorder of the NADPH oxidase characterized by severe bacterial and fungal infections and disordered inflammation. We propose that NADPH oxidase has a key role in regulating acute neutrophilic and T cell responses, which in turn restrains fungal growth and calibrates the inflammatory response to minimize injury and allergy. In this model, superoxide-induced activation of indoleamine 2,3-dioxygenase (IDO) is a central mechanism by which the optimal balance of antifungal host defense and immune tolerance occurs. This model is based on studies in mice and requires correlation in humans.
Segal
B H
BH
Departments of Medicine and Immunology, Roswell Park Cancer Institute, Buffalo, NY 14263, USA. brahm.segal@roswellpark.org
Romani
L
L
Puccetti
P
P
D000818:Animals; D001424:Bacterial Infections / Q000276:immunology*; D004195:Disease Models, Animal; D004789:Enzyme Activation; D006105:Granulomatous Disease, Chronic / Q000201:enzymology / Q000276:immunology; D006801:Humans; D007107:Immune System / Q000502:physiology; D007108:Immune Tolerance / Q000276:immunology; D050503:Indoleamine-Pyrrole 2,3,-Dioxygenase / Q000378:metabolism; D007249:Inflammation / Q000276:immunology; D009181:Mycoses / Q000276:immunology*; D019255:NADPH Oxidases / Q000378:metabolism*; D017382:Reactive Oxygen Species / Q000276:immunology; D014364:Tryptophan / Q000378:metabolism
D050503:Indoleamine-Pyrrole 2,3,-Dioxygenase; D017382:Reactive Oxygen Species; D014364:Tryptophan; D019255:NADPH Oxidases