Literature DB >> 6297635

Chronic granulomatous disease due to granulocytes with abnormal NADPH oxidase activity and deficient cytochrome-b.

R A Seger, L Tiefenauer, T Matsunaga, A Wildfeuer, P E Newburger.   

Abstract

A patient with an X-linked genetic disease resembling chronic granulomatous disease (CGD) but differing in several aspects from previously studied cases is described. The oxidase enzyme of the patient's granulocytes was normally activated, but had reduced activity as shown by an increased Michaelis constant and decreased maximum velocity of NADPH-dependent superoxide production. Cytochrome-b was undetectable in dithionite difference spectra. This CGD-like disease further implicates cytochrome-b as an important component of the microbicidal NADPH oxidase system and provides insight into its role in the enzyme complex.

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Year:  1983        PMID： 6297635

Source DB:  PubMed          Journal:  Blood        ISSN： 0006-4971            Impact factor:   22.113


  12 in total

1.  Recombinant interferon gamma augments phagocyte superoxide production and X-chronic granulomatous disease gene expression in X-linked variant chronic granulomatous disease.

Authors:  R A Ezekowitz; S H Orkin; P E Newburger
Journal:  J Clin Invest       Date:  1987-10       Impact factor: 14.808

Review 2.  Voltage-gated proton channels: molecular biology, physiology, and pathophysiology of the H(V) family.

Authors:  Thomas E DeCoursey
Journal:  Physiol Rev       Date:  2013-04       Impact factor: 37.312

3.  Expression of the X-CGD gene during induced differentiation of myeloid leukemia cell line HL-60.

Authors:  K A Barker; S H Orkin; P E Newburger
Journal:  Mol Cell Biol       Date:  1988-07       Impact factor: 4.272

4.  Effects of interferon-gamma (IFN-gamma) and tumor necrosis factor-alpha (TNF-alpha) on the expression of LFA-1 in the moderate phenotype of leukocyte adhesion deficiency (LAD).

Authors:  M T Dimanche-Boitrel; F Le Deist; A Quillet; A Fischer; C Griscelli; B Lisowska-Grospierre
Journal:  J Clin Immunol       Date:  1989-05       Impact factor: 8.317

5.  A study of 25 patients with chronic granulomatous disease: a new classification by correlating respiratory burst, cytochrome b, and flavoprotein.

Authors:  M C Bohler; R A Seger; R Mouy; E Vilmer; A Fischer; C Griscelli
Journal:  J Clin Immunol       Date:  1986-03       Impact factor: 8.317

6.  Long polymerase chain reaction-based fluorescence in situ hybridization analysis of female carriers of X-linked chronic granulomatous disease deletions.

Authors:  Kelly Claire Simon; Deborah Noack; Julie Rae; John Curnutte; Shireen Sarraf; Valentin Kolev; Jan K Blancato
Journal:  J Mol Diagn       Date:  2005-05       Impact factor: 5.568

Review 7.  The impact of intestinal inflammation on the nutritional environment of the gut microbiota.

Authors:  Franziska Faber; Andreas J Bäumler
Journal:  Immunol Lett       Date:  2014-05-04       Impact factor: 3.685

Review 8.  The dynamics of gut-associated microbial communities during inflammation.

Authors:  Sebastian E Winter; Christopher A Lopez; Andreas J Bäumler
Journal:  EMBO Rep       Date:  2013-03-12       Impact factor: 8.807

9.  Mutations in the promoter region of the gene for gp91-phox in X-linked chronic granulomatous disease with decreased expression of cytochrome b558.

Authors:  P E Newburger; D G Skalnik; P J Hopkins; E A Eklund; J T Curnutte
Journal:  J Clin Invest       Date:  1994-09       Impact factor: 14.808

10.  Deficiency of the adhesive protein complex lymphocyte function antigen 1, complement receptor type 3, glycoprotein p150,95 in a girl with recurrent bacterial infections. Effects on phagocytic cells and lymphocyte functions.

Authors:  A Fischer; R Seger; A Durandy; B Grospierre; J L Virelizier; F Le Deist; C Griscelli; E Fischer; M Kazatchkine; M C Bohler
Journal:  J Clin Invest       Date:  1985-12       Impact factor: 14.808
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