Abdelwahab 2018 Oncogene

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Publications in the MiPMap
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.

» PMID: 30573768 Open Access

Abdelwahab EMM, Pal S, Kvell K, Sarosi V, Bai P, Rue R, Krymskaya V, McPhail D, Porter A, Pongracz JE (2018) Oncogene

Abstract: 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.


Bioblast editor: Plangger M


Labels: MiParea: Respiration, mtDNA;mt-genetics, nDNA;cell genetics, Pharmacology;toxicology  Pathology: Other 

Organism: Human  Tissue;cell: Lung;gill, Endothelial;epithelial;mesothelial cell  Preparation: Intact cells 


Coupling state: ROUTINE  Pathway: ROX  HRR: Oxygraph-2k 

2019-01