Altered Oxidative Metabolism, Motility, and Adherence in Phagocytic Cells from Cystinotic Children
Altered Oxidative Metabolism, Motility, and Adherence in Phagocytic Cells from Cystinotic Children
复制标题
胱氨酸病儿童吞噬细胞氧化代谢、运动和粘附的改变
DOI：
10.1203/00006450-198512000-00023
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发表时间：
1985
影响因子：
3.6
通讯作者：
B. Descamps
中科院分区：
文献类型：
--
作者：
Guillem Pintos Morell;P. Niaudet;G. Jean;B. Descamps
ABSTRACT: The present study investigates whether the metabolic abnormalities in cystinotic cells could affect nonspecific immune responses. Lymphocytes showed normal antibody-dependent cellular cytotoxicity and natural killer activity. However, cystinotic polymorphonuclear and mononuclear phagocytes exhibited altered oxidative responses as monitored by a luminol dependent chemiluminescence (CL) assay. Both isolated polymorphonuclear and mononuclear phagocytes in the absence of stimuli showed significantly increased CL production which was not found when cells were tested directly within whole blood. CL responses to a panel of stimuli differed markedly according to the type of cells and agents tested. Indeed, isolated polymorphonuclear demonstrated increased CL responses to soluble but not particulate agents, whereas isolated mononuclear phagocytes and overall cell CL responses in whole blood were found to be within the normal range regardless of the type of stimulus used. We also studied some membrane related properties of phagocytic cells. Fc and C3b receptors were normally expressed as tested by erythrocyte-antibody and erythrocyte-antibody-complement rosette-forming cells. Nevertheless, cystinotic polymorphonuclear and mononuclear phagocytes presented decreased random and directed migrations in an under-agarose chemotaxis assay. Finally, cystinotic granulocytes showed an impaired adhesiveness in a nylon fiber assay.
胱氨酸中毒时，ATP 依赖性溶酶体胱氨酸流出存在缺陷。
DOI：
--
发表时间：
1982
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Jonas,AJ;Smith,ML;Schneider,JA
通讯作者：
Schneider,JA