Human pluripotent stem cell-derived TSC2-haploinsufficient smooth muscle cells recapitulate features of Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a progressive destructive neoplasm of the lung associated with inactivating mutations in the TSC1 or TSC2 tumor suppressor genes. Cell or animal models that accurately reflect the pathology of LAM have been challenging to develop. Here we generated a robust human ce...

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Bibliographic Details
Published in:Cancer Res
Main Authors: Julian, Lisa M., Delaney, Sean P., Wang, Ying, Goldberg, Alexander A., Doré, Carole, Yockell-Lelièvre, Julien, Tam, Roger Y., Giannikou, Krinio, McMurray, Fiona, Shoichet, Molly S., Harper, Mary-Ellen, Henske, Elizabeth P., Kwiatkowski, David J., Darling, Thomas N., Moss, Joel, Kristof, Arnold S., Stanford, William L.
Format: Artigo
Language:Inglês
Published: 2017
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC5645248/
https://ncbi.nlm.nih.gov/pubmed/28830860
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1158/0008-5472.CAN-17-0925
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