scholarly journals Treatment of patients with chronic granulomatous disease with recombinant human interferon-gamma does not improve neutrophil oxidative metabolism, cytochrome b558 content or levels of four anti-microbial proteins

2008 ◽  
Vol 88 (2) ◽  
pp. 203-206 ◽  
Author(s):  
T. J. MÜHLEBACH ◽  
J. GABAY ◽  
C.F. NATHAN ◽  
C. ERNY ◽  
G. DOPFER ◽  
...  
1996 ◽  
Vol 315 (2) ◽  
pp. 571-575 ◽  
Author(s):  
Colin D. PORTER ◽  
KURIBAYASHI KURIBAYASHI ◽  
Mohamed H. PARKAR ◽  
Dirk ROOS ◽  
Christine KINNON

NADPH oxidase cytochrome b558 consists of two subunits, gp91-phox and p22-phox, defects of which result in chronic granulomatous disease (CGD). The nature of the interaction between these subunits has yet to be determined. Absence of p22-phox in autosomal CGD patient-derived B-cell lines results in detectable levels of an incompletely glycosylated gp91-phox precursor. We have detected this same precursor species in four cell lines from patients with the X-linked form of the disease due to mutations in gp91-phox. Such mutations should delineate regions of gp91-phox important for its biosynthesis, including stable association with p22-phox. One mutation mapped to the putative FAD-binding domain, one mapped to a potential haem-binding domain, and two involved the region encoded by exon 3.


PEDIATRICS ◽  
1990 ◽  
Vol 85 (2) ◽  
pp. 219-221
Author(s):  
REBECCA J. COLLMAN ◽  
JOSEPH D. DICKERMAN

Chronic granulomatous disease refers to a group of inherited disorders characterized by a deficit in phagocytic-cell oxidative metabolism, resulting in recurrent pyogenic infections due to defective intracellular killing of microorganisms.1 Granulomas form in various organs or tissues, presumably in an attempt to "wall off" the infection, and these can be a major cause of morbidity when vital structures are obstructed or compromised. Cultures of the granulomas are usually negative, although antibiotics have been advocated in the treatment of this complication. Recently, three patients with chronic granulomatous disease-two with gastrointestinal and genitourinary involvement2 and one with pulmonary involvement3—were treated with corticosteroids, which resulted in an amelioration of symptoms.


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