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Published ahead of print on June 30, 2005, doi:10.1165/rcmb.2005-0152RC
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American Journal of Respiratory Cell and Molecular Biology. Vol. 33, pp. 227-230, 2005
© 2005 American Thoracic Society
DOI: 10.1165/rcmb.2005-0152RC

Rapid Communication

Interferon-{gamma}-Jak-Stat Signaling in Pulmonary Lymphangioleiomyomatosis and Renal Angiomyolipoma

A Potential Therapeutic Target

Nisreen El-Hashemite and David J. Kwiatkowski

Brigham and Women's Hospital, Hematology Division, Department of Medicine, Harvard Medical School, Boston, Massachusetts

Correspondence and requests for reprints should be addressed to David J. Kwiatkowski, Brigham and Women's Hospital, Hematology Division, Department of Medicine, Harvard Medical School, 75 Francis Street, Boston MA 02115. E-mail: dk{at}rics.bwh.harvard.edu

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.

Key Words: angiomyolipoma • interferon-{gamma} • lymphangioleiomyomatosis • Stats • tuberous sclerosis




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