Vowells S J, Fleisher T A, Sekhsaria S, Alling D W, Maguire T E, Malech H L
Laboratory of Host Defenses, National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, Maryland 20892, USA.
J Pediatr. 1996 Jan;128(1):104-7. doi: 10.1016/s0022-3476(96)70437-7.
We studied phagocyte reduced nicotinamide adenine dinucleotide phosphate function to evaluate production of reactive oxygen species in both X-linked and autosomal forms of chronic granulomatous disease. We found a consistent and significant difference between the activated granulocyte response of the X-linked (gp91-phagocyte oxidase) form of chronic granulomatous disease (n = 18) and that of the most common autosomal recessive (p47-phagocyte oxidase) form of the disease (n = 17). The data indicate that mutations in the p47-phagocyte oxidase component of the reduced nicotinamide adenine dinucleotide phosphate oxidase component do not completely prevent oxidation despite severe defects in superoxide generation.
我们研究了吞噬细胞还原型烟酰胺腺嘌呤二核苷酸磷酸功能，以评估X连锁和常染色体形式的慢性肉芽肿病中活性氧的产生。我们发现，X连锁（gp91-吞噬细胞氧化酶）形式的慢性肉芽肿病（n = 18）与最常见的常染色体隐性（p47-吞噬细胞氧化酶）形式的慢性肉芽肿病（n = 17）的活化粒细胞反应之间存在一致且显著的差异。数据表明，还原型烟酰胺腺嘌呤二核苷酸磷酸氧化酶成分中p47-吞噬细胞氧化酶成分的突变尽管在超氧化物生成方面存在严重缺陷，但并未完全阻止氧化。
