自身免疫淋巴增生性免疫缺陷病部分患儿病情重,预后差,严重威胁患儿生命。该病的发病机制主要分为两类:第一类为FAS通路相关基因突变导致淋巴细胞凋亡障碍,过多未凋亡细胞在淋巴系统聚集形成淋巴增殖表现;第二类是由磷脂酰肌醇3-激酶/...自身免疫淋巴增生性免疫缺陷病部分患儿病情重,预后差,严重威胁患儿生命。该病的发病机制主要分为两类:第一类为FAS通路相关基因突变导致淋巴细胞凋亡障碍,过多未凋亡细胞在淋巴系统聚集形成淋巴增殖表现;第二类是由磷脂酰肌醇3-激酶/蛋白激酶B/哺乳动物雷帕霉素靶蛋白[(phosphoinositide 3-kinase/protein kinase B(PKB/Akt)/mammalian target of rapamycin,PI3K/Akt/mTOR)]、RAS/丝裂原活化蛋白激酶(RAS/mitogen-activated protein kinase,RAS/MAPK)等信号通路过度活化造成淋巴细胞过度活化增殖,增殖的淋巴细胞聚集而引起。展开更多
Objective.To investigate the effect of peroxis ome proliferator-activated recept ors(PPARs )activators on plasminogen activator inhibitor ty pe-1(PAI-1)expression in human umbilical vein e ndothelial cells and the pos...Objective.To investigate the effect of peroxis ome proliferator-activated recept ors(PPARs )activators on plasminogen activator inhibitor ty pe-1(PAI-1)expression in human umbilical vein e ndothelial cells and the possi-ble mechanism.Methods.Human umbilical vein endothelial ce lls(HUVECs )were obtained from normal fetus,and cul-tured conventionally.Then the HUVECs were exposed to test agents(linolenic acid,linoleic acid,oleic acid,stearic acid and prostaglandin J 2 respectively)in varying concentrations with fresh media.RT -PCR and ELISA were applied to determine the expression of PPARs and PAI-1in HUVECs.Results.PPARα,PPARδand PPARγmRNA were detected by using RT-PCR in HUVECs.Treatment of HUVECs with PPARαand PPARγactivators---linolenic acid,linoleic acid,oleic acid and prostaglandin J 2 respectively,but not with stearic a cid could augment PAI-I mRNA expression and protein secretion in a concentration-dependent manner.However,the mRNA expressions of 3subclasses of PPAR with their activators in HUVECs were not changed compared w ith controls.Conclusion.HUVECs express PPARs.PPARs activators may increase PAI-1expression in ECs,but the underlying mechanism remains uncle ar.Although PPARs expression was not enhanced after stimulated by their activators in ECs,the role of functionally active PPARs in regulating PA I-1expression in ECs needs to be further investigated by using transient gen e transfection assay.展开更多
文摘自身免疫淋巴增生性免疫缺陷病部分患儿病情重,预后差,严重威胁患儿生命。该病的发病机制主要分为两类:第一类为FAS通路相关基因突变导致淋巴细胞凋亡障碍,过多未凋亡细胞在淋巴系统聚集形成淋巴增殖表现;第二类是由磷脂酰肌醇3-激酶/蛋白激酶B/哺乳动物雷帕霉素靶蛋白[(phosphoinositide 3-kinase/protein kinase B(PKB/Akt)/mammalian target of rapamycin,PI3K/Akt/mTOR)]、RAS/丝裂原活化蛋白激酶(RAS/mitogen-activated protein kinase,RAS/MAPK)等信号通路过度活化造成淋巴细胞过度活化增殖,增殖的淋巴细胞聚集而引起。
文摘Objective.To investigate the effect of peroxis ome proliferator-activated recept ors(PPARs )activators on plasminogen activator inhibitor ty pe-1(PAI-1)expression in human umbilical vein e ndothelial cells and the possi-ble mechanism.Methods.Human umbilical vein endothelial ce lls(HUVECs )were obtained from normal fetus,and cul-tured conventionally.Then the HUVECs were exposed to test agents(linolenic acid,linoleic acid,oleic acid,stearic acid and prostaglandin J 2 respectively)in varying concentrations with fresh media.RT -PCR and ELISA were applied to determine the expression of PPARs and PAI-1in HUVECs.Results.PPARα,PPARδand PPARγmRNA were detected by using RT-PCR in HUVECs.Treatment of HUVECs with PPARαand PPARγactivators---linolenic acid,linoleic acid,oleic acid and prostaglandin J 2 respectively,but not with stearic a cid could augment PAI-I mRNA expression and protein secretion in a concentration-dependent manner.However,the mRNA expressions of 3subclasses of PPAR with their activators in HUVECs were not changed compared w ith controls.Conclusion.HUVECs express PPARs.PPARs activators may increase PAI-1expression in ECs,but the underlying mechanism remains uncle ar.Although PPARs expression was not enhanced after stimulated by their activators in ECs,the role of functionally active PPARs in regulating PA I-1expression in ECs needs to be further investigated by using transient gen e transfection assay.
