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Abdelwahab 2018 Oncogene
Additional label 2019-01  +
Coupling states ROUTINE  +
Diseases Other  +
Has abstract Lymphangioleiomyomatosis (LAM) is a rare a
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.
ells provide a novel target for treatment.  +
Has editor [[Plangger M]]  +
Has info [ PMID: 30573768 Open Access]  +
Has title Abdelwahab EMM, Pal S, Kvell K, Sarosi V,
Abdelwahab EMM, Pal S, Kvell K, Sarosi V, Bai P, Rue R, Krymskaya V, McPhail D, Porter A, Pongracz JE (2018) Mitochondrial dysfunction is a key determinant of the rare disease lymphangioleiomyomatosis and provides a novel therapeutic target. Oncogene 38:3093-3101.
therapeutic target. Oncogene 38:3093-3101.  +
Instrument and method Oxygraph-2k  +
Mammal and model Human  +
MiP area Respiration  + , mtDNA;mt-genetics  + , nDNA;cell genetics  + , Pharmacology;toxicology  +
Pathways ROX  +
Preparation Intact cells  +
Tissue and cell Lung;gill  + , Endothelial;epithelial;mesothelial cell  +
Was published in journal Oncogene +
Was published in year 2018  +
Was written by Abdelwahab EMM + , Pal S + , Kvell K + , Sarosi V + , Bai P + , Rue R + , Krymskaya V + , McPhail D + , Porter A + , Pongracz JE +
Categories Publications
Modification date
"Modification date" is a predefined property that corresponds to the date of the last modification of a subject and is provided by Semantic MediaWiki.
10:32:49, 9 August 2019  +
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