RAPID COMMUNICATION Restoration of Superoxide Generation to a Chronic Granulomatous Disease-Derived B-Cell Line by Retrovirus Mediated Gene Transfer
نویسندگان
چکیده
Failure of a superoxide generating system, the NADPH oxidase, present in neutrophils and other phagocytes gives rise to chronic granulomatous disease (CGD), a group of singlegene inherited disorders all characterized by an extreme susceptibility to pyogenic infection, with potentially fatal consequences. About 30% of CGD cases are caused by an autosomally inherited deficiency of a 47-Kd cytoplasmic component of the oxidase (p47-phox). Epstein-Barr virus (EBV) immortalized 8-lymphocyte lines established from
منابع مشابه
Restoration of superoxide generation to a chronic granulomatous disease-derived B-cell line by retrovirus mediated gene transfer.
Failure of a superoxide generating system, the NADPH oxidase, present in neutrophils and other phagocytes gives rise to chronic granulomatous disease (CGD), a group of single-gene inherited disorders all characterized by an extreme susceptibility to pyogenic infection, with potentially fatal consequences. About 30% of CGD cases are caused by an autosomally inherited deficiency of a 47-Kd cytopl...
متن کاملX-linked chronic granulomatous disease: correction of NADPH oxidase defect by retrovirus-mediated expression of gp91-phox.
Chronic granulomatous disease (CGD) is an inherited immunodeficiency resulting from the inability of an individual's phagocytes to produce superoxide anions because of defective NADPH oxidase. The disease may be treated by bone marrow transplantation and as such is a candidate for somatic gene therapy. Two thirds of patients have defects in an X-linked gene (X-CGD) encoding gp91-phox, the large...
متن کاملRAPID COMMUNICATION CD34+ Peripheral Blood Progenitors as a Target for Genetic Correction of the Two Flavocytochrome bSs8 Defective Forms of Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) can result from any of four single gene defects involving components of the superoxide (0;')-generating phagocyte NADPH oxidase (phox). The phox transmembrane flavocytochrome bsss is composed of two peptides, gpS1Ph" and ~ 2 2 ~ " " . Mutations of gp9lW" cause X-linked CGD, whereas mutations of p22pho" cause one of the three autosomal recessive forms of CGD....
متن کاملGenetic Correction of p67 Deficient Chronic Granulomatous Disease Using Peripheral Blood Progenitor Cells as a Target for Retrovirus Mediated Gene Transfer
Chronic granulomatous disease (CGD) can result from any from transduced CD34 progenitors from the p67 CGD patient were oxidase positive with the average level of corof four single gene defects involving the components of the superoxide (O2 ) generating phagocyte nicotinamide aderection per granulocyte of 85% of that seen with granulocytes in similar cultures of CD34 progenitors from normal nine...
متن کاملGenetic correction of p67phox deficient chronic granulomatous disease using peripheral blood progenitor cells as a target for retrovirus mediated gene transfer.
Chronic granulomatous disease (CGD) can result from any of four single gene defects involving the components of the superoxide (O-2) generating phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. We show that transduction of peripheral blood CD34+ hematopoietic progenitors from a p67phox deficient CGD patient with replication defective amphotropic retrovirus encoding p67phox ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2003