chronic granulomatous disease (cgd) is a rare primary immunodeficiency disorder due to a genetic defect in one of the components of nicotinamide adenine dinucleotide phosphate (nadph) oxidase complex. this complex is composed of membrane-bound gp91- phox and p22- phox subunits, and cytosolic subunits consisting of p47- phox , p67- phox , and p40- phox . a mutation in cybb gene encoding gp91- ph...

activity and mRNA expression (NOX1, NOX4, p67, p47, p22). Inhibitors of AP-1 significantly diminished the Ang II or TNF-stimulated p22 promoter activity and protein level. Transient overexpression of c-Jun/c-Fos upregulated p22 promoter activity. Transcription factor pull-down assay and chromatin immunoprecipitation demonstrated the physical interaction of c-Jun protein with predicted AP-1–bind...

A 12 year old boy suffering from p67-phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal papillae. By direct immunofluorescence, linear staining of IgA at the dermal-epidermal junction w...

Abstract For about 2 months (from 13 January to 15 March 2022) our CICU–Cardiology department (spoke for the AMI network) of "San Giovanni di Dio" Hospital Frattamaggiore (Napoli) has assumed role Covid–CICU ASL Napoli Nord. We analyzed data regarding patients hospitalized acute myocardial infarction in from August, selected on basis presence SARS–COV2 infection at admission, and we noted some ...

We present the first functional analysis of the small GTPase, TbRab7, in Trypanosoma brucei. TbRab7 defines discrete late endosomes closely juxtaposed to the terminal p67(+) lysosome. RNAi indicates that TbRab7 is essential in bloodstream trypanosomes. Initial rates of endocytosis were unaffected, but lysosomal delivery of cargo, including tomato lectin (TL) and trypanolytic factor (TLF) were ...

Ultraviolet A (UVA) radiation, the major UV component of solar radiation, can penetrate easily to the dermis, where it causes significant damage to cellular components by inducing formation of reactive oxygen species (ROS). On the other hand, extracellular ATP is released in response to various stimuli, and activates purinergic P2X7 receptor, triggering ROS production and cell death. Here, we e...

BACKGROUND: Cancer cachexia is the progressive loss of skeletal muscle protein that contributes significantly to cancer morbidity and mortality. Evidence of antioxidant attenuation and the presence of oxidised proteins in patients with cancer cachexia indicate a role for oxidative stress. The level of oxidative stress in tissues is determined by an imbalance between reactive oxygen species prod...

Bacterial LPS is a pluripotent agonist for PMNs. Although it does not activate the NADPH-dependent oxidase directly, LPS renders PMNs more responsive to other stimuli, a phenomenon known as "priming." Since the mechanism of LPS-dependent priming is incompletely understood, we investigated its effects on assembly and activation of the NADPH oxidase. LPS pretreatment increased superoxide (O2-) ge...

Studies in Chronic Granulomatous Disease showed two breakthroughs during this past decade. First, the discovery of 7 Nox/Duox family proteins, Noxo1 and Noxa1 (homologues of gp91(phox), p47(phox) and p67(phox)) may clarify novel physiological mechanisms for superoxide regulation in various organs, such as the regulation of blood pressure, mucosal defense system in respiratory/digestive tract an...