Logo image
Proapoptotic protein Bim attenuates estrogen-enhanced survival in lymphangioleiomyomatosis
Journal article   Open access   Peer reviewed

Proapoptotic protein Bim attenuates estrogen-enhanced survival in lymphangioleiomyomatosis

Chenggang Li, Na Li, Xiaolei Liu, Erik Y Zhang, Yang Sun, Kouhei Masuda, Jing Li, Julia Sun, Tasha Morrison, Xiangke Li, …
JCI insight, v 1(19), pp e86629-e86629
17 Nov 2016
PMID: 27882343
url
https://doi.org/10.1172/jci.insight.86629View
Published, Version of Record (VoR) Open

Abstract

Estrogens - physiology Humans Bortezomib - pharmacology Lung - cytology Lymphangioleiomyomatosis - pathology Mice, SCID Animals Bcl-2-Like Protein 11 - metabolism Tuberous Sclerosis Complex 2 Protein Tumor Suppressor Proteins - genetics Anoikis Female Mice Lung Diseases - pathology Tumor Cells, Cultured
Lymphangioleiomyomatosis (LAM) is a progressive lung disease that primarily affects young women. Genetic evidence suggests that LAM cells bearing mutations migrate to the lungs, proliferate, and cause cystic remodeling. The female predominance indicates that estrogen plays a critical role in LAM pathogenesis, and we have proposed that estrogen promotes LAM cell metastasis by inhibition of anoikis. We report here that estrogen increased LAM patient-derived cells' resistance to anoikis in vitro, accompanied by decreased accumulation of the proapoptotic protein Bim, an activator of anoikis. The resistance to anoikis was reversed by the proteasome inhibitor, bortezomib. Treatment of LAM patient-derived cells with estrogen plus bortezomib promoted anoikis compared with estrogen alone. Depletion of Bim by siRNA in TSC2-deficient cells resulted in anoikis resistance. Treatment of mice with bortezomib reduced estrogen-promoted lung colonization of TSC2-deficient cells. Importantly, molecular depletion of Bim by siRNA in Tsc2-deficient cells increased lung colonization in a mouse model. Collectively, these data indicate that Bim plays a key role in estrogen-enhanced survival of LAM patient-derived cells under detached conditions that occur with dissemination. Thus, targeting Bim may be a plausible future treatment strategy in patients with LAM.

Metrics

8 Record Views
8 citations in Scopus

Details

UN Sustainable Development Goals (SDGs)

This publication has contributed to the advancement of the following goals:

#3 Good Health and Well-Being

InCites Highlights

Data related to this publication, from InCites Benchmarking & Analytics tool:

Collaboration types
Domestic collaboration
International collaboration
Web of Science research areas
Medicine, Research & Experimental
Logo image