Article
Molecular quality control machinery contributes to the leukocyte NADPH oxidase deficiency in chronic granulomatous disease.
Biochimica et biophysica acta - 24 Apr 2002
Lin Shio-Jean, Huang Ya-Fang, Chen Jing-Yi, Heyworth Paul G, Noack Deborah, Wang Ji-Yao, Lin Ching-Yuan, Chiang Bor-Luen, Yang Chin-Mu, Liu Ching-Chuan, Shieh Chi-Chang
Abstract excerpt
Chronic granulomatous disease (CGD) is an inherited immunodeficiency disease caused by defects in leukocyte NADPH oxidase. Various inherited defects in one of the membrane-bound components of NADPH oxidase, gp91-phox, cause X-linked (X91) CGD. Analysis of three patients with X91 CGD revealed that different mechanisms of molecular quality control lead to the common phenotype of absence of mature membrane-bound...
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