PHAGOCYTIC CELL FUNCTION

这个项目的主要目标是研究中性粒细胞的功能。 在健康和疾病方面。我们已经证明，先天缺陷的患者 在中性粒细胞中，特殊颗粒在中性粒细胞中有主要的异常。 功能。正常中性粒细胞FMet-Leu-Phe受体和C3bi受体 (CT3)大量存在于细胞内池中， 蔗糖与正常中性粒细胞特定颗粒的复合制剂 和Percoll渐变。这些细胞被转移到细胞表面 神经细胞的激活导致FMET-Leu-Phe增加三倍 受体和CR3。CR3缺乏症患者中性粒细胞异常 趋化性、粘附性和扩散性。比较外周血的研究 在豚鼠和人身上的实验渗出细胞表明 渗出液中有中性粒细胞特异性颗粒优先丢失。 细胞。此外，渗出细胞还增加了fMet-leu-Phe和C3bi 受体表达。细胞色素b存在于特定的颗粒中，是一种 电子传输链的重要组成部分 超氧化物生产。我们已经证明，中性粒细胞的激活 呼吸爆发需要细胞色素b从细胞内移位 被钙离子载体激活的质膜隔室 A23187，但不能被佛波醇酯和FMET-Leu-Phe激活。共30个 11例慢性肉芽肿患者细胞色素b缺乏 (10名男孩有X连锁遗传，但有趣的是有一名患者 是一个可能有常染色体隐性遗传的女孩。在其他研究中 在高IgE综合征(反复感染)患者中，我们发现 在这些患者中，IgE分解代谢降低。

The major objective of this project has been to study neutrophil function in health and disease. We have shown that a patient congenitally deficient in neutrophil specific granules has major abnormalities in neutrophil function. In normal neutrophils fmet-leu-phe receptors and C3bi receptors (CT3) are present in large amounts in an intracellular pool that cosediments with the specific granules from normal neutrophils on sucrose and Percoll gradients. These are translocated to the cell surface after activation of neurophils resulting in a three fold increase in fmet-leu-phe receptor and CR3. CR3 deficient patients' neutrophils have abnormal chemotaxis, adhesiveness and spreading. Studies comparing peripheral blood and experimental exudate cells in guinea pigs and in man it has shown that there is preferential loss of neutrophil specific granules in the exudate cells. In addition, exudate cells have incresed fmet-leu-phe and C3bi receptor expression. Cytochrome b, found in specific granules, is an important component of the electron transport chain essential for superoxide production. We have shown that neutrophil activation of the respiratory burst requires cytochrome b translocation from an intracellular compartment to the plasma membrane with activation by the calcium ionophore A23187 but not with activation by phorbol esters and fmet-leu-phe. Of 30 patients with chronic granulomatous disease, 11 had cytochrome b deficiency (10 were boys with an X-linked inheritance but interestingly one patient was a girl with probable autosomal recessive inheritance. In other studies of patients with hyper IgE syndrome (recurrent infections) we found that there is decrease catabolism of IgE in these patients.