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FIBROSIS SIGNED

Fibroproliferative Invasive Fibroblasts in Idiopathic Pulmonary Fibrosis

Total Cost €

EC-Contrib. €

Partnership

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FIBROSIS project word cloud

Explore the words cloud of the FIBROSIS project. It provides you a very rough idea of what is the project "FIBROSIS" about.

fibroblasts technologies restoration ireland diseases dysregulated rare disease augmenting regions lung relentlessly multiple respiratory generate phenotype injured elusive lineage central source fibroblast delineate alveolar cedars sinai gas disciplines interdisciplinary mouse invasive uniquely progressive rna cutting die sequencing pathogenic researcher donors usa world human skills discovered centre merge lethal healing structure idiopathic medical accumulation single cell exchange acquired diagnostic characterisation highlighted genetically patients international prognosis pulmonary 5years basis incidence model diagnosis destruction dublin survival normal vitro ipf prof applicable tracing university mcloughlin therapies college noble fibrogenesis latest molecular wound explore fibrosis modified unknown paul function edge poor

Project "FIBROSIS" data sheet

The following table provides information about the project.

Coordinator	UNIVERSITY COLLEGE DUBLIN, NATIONAL UNIVERSITY OF IRELAND, DUBLIN Organization address address: BELFIELD city: DUBLIN postcode: 4 website: www.ucd.ie contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.
Coordinator Country	Ireland [IE]
Total cost	266˙063 €
EC max contribution	266˙063 € (100%)
Programme	1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
Code Call	H2020-MSCA-IF-2017
Funding Scheme	MSCA-IF-GF
Starting year	2018
Duration (year-month-day)	from 2018-09-03 to 2021-09-02

Partnership

Take a look of project's partnership.

#	participants	country	role	EC contrib. [€]
1	UNIVERSITY COLLEGE DUBLIN, NATIONAL UNIVERSITY OF IRELAND, DUBLIN UNIVERSITY COLLEGE DUBLIN, NATIONAL UNIVERSITY OF IRELAND, DUBLIN Organization address address: BELFIELD city: DUBLIN postcode: 4 website: www.ucd.ie contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	IE (DUBLIN)	coordinator	266˙063.00
2	CEDARS-SINAI MEDICAL CENTER CEDARS-SINAI MEDICAL CENTER Organization address address: BEVERLY BOULEVARD 8700 city: LOS ANGELES CA postcode: 90048 website: www.cedars-sinai.edu contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	US (LOS ANGELES CA)	partner	0.00

Map

Project objective

Idiopathic Pulmonary Fibrosis (IPF) is a relentlessly progressive, lethal, lung disease with a uniquely poor prognosis. Patients typically die within 2-5years of diagnosis and IPF’s European incidence is increasing while effective therapies for improving survival remain elusive.

IPF is a disease in which the normal wound healing responses are dysregulated leading to fibrosis and loss of gas-exchange regions in the lung rather than restoration of normal lung structure and function. Recently discovered invasive fibroblasts are believed to be central to the progressive fibrogenesis observed in IPF by contributing to an accumulation of fibroblasts in the injured lung and augmenting the destruction of normal alveolar structure. However the source of these invasive fibroblasts and what makes them so uniquely pathogenic is unknown.

On this project I will work with Prof. Paul Noble, Cedars-Sinai Medical Centre, USA, one of the world’s leading IPF researchers and Ireland’s leading respiratory researcher Prof. Paul McLoughlin, University College Dublin, Ireland. The objectives of this project are to investigate: (1) the phenotype and behaviour of the invasive fibroblast from human IPF donors in vitro; (2) delineate the molecular pathways that make invasive human IPF fibroblasts uniquely pathogenic using state-of-the-art single cell RNA-sequencing; (3) determine the source of invasive fibroblast accumulation in the IPF lung using the most advanced lineage tracing methods available in a cutting-edge genetically modified mouse model.

This interdisciplinary proposal will merge skills I have acquired with the latest technologies across multiple disciplines in world leading international institutions to explore the complex molecular basis of IPF. The results of this proposal will generate significant impact in the IPF field and will be directly applicable to the specific challenge highlighted in the H2020 Work Programme: Diagnostic characterisation of rare diseases.

Publications

List of publications.
year	authors and title	journal	last update
2019	Simon Coyle Rowan, Stephanie Bora, Ankita Burman, Ting Xie, Peter Chen Recommended Reading from Cedars-Sinai Medical Center Fellows published pages: , ISSN: 1044-1549, DOI: 10.1165/rcmb.2019-0196ro	American Journal of Respiratory Cell and Molecular Biology	2019-10-15

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The information about "FIBROSIS" are provided by the European Opendata Portal: CORDIS opendata.