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Interferon-gamma-Jak-Stat signaling in pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a potential therapeutic target.

Authors: El-Hashemite, N  Kwiatkowski, DJ 
Citation: El-Hashemite N and Kwiatkowski DJ, Am J Respir Cell Mol Biol. 2005 Sep;33(3):227-30. Epub 2005 Jun 30.
Pubmed: (View Article at PubMed) PMID:15994429
DOI: Full-text: DOI:10.1165/rcmb.2005-0152RC

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.


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CRRD Object Information
CRRD ID: 2298537
Created: 2008-07-09
Species: All species
Last Modified: 2008-07-09
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.