Journal
ONCOGENE
Volume 38, Issue 16, Pages 3093-3101Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/s41388-018-0625-1
Keywords
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Funding
- European Union
- State of Hungary - European Social Fund [TAMOP-4.2.4, A/2-11/1-2012-0001]
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Lymphangioleiomyomatosis (LAM) is a rare and progressive systemic disease affecting mainly young women of childbearing age. A deterioration in lung function is driven by neoplastic growth of atypical smooth muscle-like LAM cells in the pulmonary interstitial space that leads to cystic lung destruction and spontaneous pneumothoraces. Therapeutic options for preventing disease progression are limited and often end with lung transplantation temporarily delaying an inevitable decline. To identify new therapeutic strategies for this crippling orphan disease, we have performed array based and metabolic molecular analysis on patient-derived cell lines. Our results point to the conclusion that mitochondrial biogenesis and mitochondrial dysfunction in LAM cells provide a novel target for treatment.
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