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PLoS One. 2014 Aug 18;9(8):e105365. doi: 10.1371/journal.pone.0105365. eCollection 2014.

A quantitative proteomic approach to identify significantly altered protein networks in the serum of patients with lymphangioleiomyomatosis (LAM).

Author information

1
Woolcock Institute of Medical Research, The University of Sydney, Sydney, NSW, Australia.
2
Woolcock Institute of Medical Research, The University of Sydney, Sydney, NSW, Australia; Discipline of Pharmacology, The University of Sydney, Sydney, NSW, Australia.
3
University of Southampton, Southampton, United Kingdom.
4
Center for Rare Lung Disease, University of Modena and Reggio Emilia, Modena, Italy.
5
Woolcock Institute of Medical Research, The University of Sydney, Sydney, NSW, Australia; School of Medical and Molecular Biosciences, University of Technology, Sydney, NSW, Australia.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung condition affecting approximately 3.4-7.5/million women, with an average lag time between symptom onset and diagnosis of upwards of 4 years. The aim of this work was to identify altered proteins in LAM serum which may be potential biomarkers of disease. Serum from LAM patient volunteers and healthy control volunteers were pooled and analysis carried out using quantitative 4-plex iTRAQ technology. Differentially expressed proteins were validated using ELISAs and pathway analysis was carried out using Ingenuity Pathway Analysis. Fourteen proteins were differentially expressed in LAM serum compared to control serum (p<0.05). Further screening validated the observed differences in extracellular matrix remodelling proteins including fibronectin (30% decrease in LAM, p = 0.03), von Willebrand Factor (40% reduction in LAM, p = 0.03) and Kallikrein III (25% increase in LAM, p = 0.03). Pathway networks elucidated the relationships between the ECM and cell trafficking in LAM. This study was the first to highlight an imbalance in networks important for remodelling in LAM, providing a set of novel potential biomarkers. These understandings may lead to a new effective treatment for LAM in the future.

PMID:
25133674
PMCID:
PMC4136818
DOI:
10.1371/journal.pone.0105365
[Indexed for MEDLINE]
Free PMC Article

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