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Cytochrome b consists of two sub-units gp91 phox and p22 phox. In some rare cases, the mutated gp91 phox is normally expressed but is devoided of oxidase activity.
However modelization of such variants is necessary to obtain enough biological material for studies at the molecular level. A cellular model knock-out PLB cells has been developed for expressing recombinant mutated gp91 phox for functional analysis of the oxidase complex. Recent works demonstrated that this cell line genetically deficient in gp91 phox is a powerful tool for functional analysis of the NADPH oxidase complex activation.
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Genetics and Immunopathology of Chronic Granulomatous Disease
E-mail :. Nous en rapportons une observation. Chronic septic granulomatosis is a disease characterized by an impaired bactericidal potential of the neutrophilic polynuclear. The cutaneous manifestations rarely reveal the disease, but are of considerable interest in the diagnosis, notably during the late onset forms. We report such a case. A 15 year-old girl, born of consanguine parents, had a history of visceral leishmaniasis and hepatic hydatidosis.
Translation of "granulomatose septique" in English
Cytochrome b consists of two sub-units gp91 phox and p22 phox. In some rare cases, the mutated gp91 phox is normally expressed but is devoided of oxidase activity. However modelization of such variants is necessary to obtain enough biological material for studies at the molecular level. A cellular model knock-out PLB cells has been developed for expressing recombinant mutated gp91 phox for functional analysis of the oxidase complex.
P-295 – Granulomatose septique chronique au Maroc
The chronic granulomatous disease CGD is a rare immunodeficiency, consecutive to a defect of the oxidative metabolism of phagocyt. We reviewed the epidemiologic, clinical, biological and therapeutic features of eight cases of CGD. Average age of the beginning of the symptomatology is of 8. Clinical manifestations are often infections bacterial or fungal in lung. We report four cases of invasive pulmonary aspergillosis.
Chronic-granulomatous disease CGD is a rare inherited primary immunodeficiency syndrome caused by a defective oxidative metabolism of phagocytic cells. Dysfunction of the membranous NADPH oxidase complex leads to a greatly increased susceptibility to severe fungal and bacterial infections, early in childhood. The most severe and frequent type of GCD is the X-linked transmitted form caused by mutations in the CYB B gene encoding the redox element of the oxidase complex, gp91phox or Nox2. However, very rare autosomal recessive CGD affecting other oxidase components than Nox2 are characterized by mild-clinical manifestations that could appear later at the adult age.