Interferon-γ-Jak-Stat signaling in pulmonary lymphangioleiomyomatosis and renal anglomyolipoma -: A potential therapeutic target

被引:30
作者
El-Hashemite, N [1 ]
Kwiatkowski, DJ [1 ]
机构
[1] Harvard Univ, Sch Med, Brigham & Womens Hosp, Dept Med,Div Hematol, Boston, MA 02115 USA
关键词
angiomyolipoma; interferon-gamma; lymphangioleiomyomatosis; Stats; tuberous sclerosis;
D O I
10.1165/rcmb.2005-0152RC
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML) are proliferative lesions that occur in sporadic patients, and at much higher frequency in patients with tuberous sclerosis (TSC). The TSC1 and TSC2 genes play a critical role in their pathogenesis. Here we report a marked decrease in interferon (IFN)-gamma expression in both sporadic and TSC-associated AML and LAM. A marked increase in Stat1 expression and phosphorylation at Ser 727, and in phospho-Tyr705-Stat3 levels, was also seen in both AML and LAM tissues. Our results demonstrate that the IFN-gamma-Jak-Stat pathway is perturbed in TSC-related and sporadic LAM and AML, and suggest that IFN-gamma has potential therapeutic benefit for treatment of those lesions.
引用
收藏
页码:227 / 230
页数:4
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