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FAtoUnFRAGILITY SIGNED

Fanconi anemia : a disease model to understand causes and consequences of common fragile site instability.

Total Cost €

EC-Contrib. €

Partnership

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Outcomes and
results

FAtoUnFRAGILITY project word cloud

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

abnormalities fragile underpinning significantly rearrange bone featuring examine critical proliferation understand altering exploring strategies propensity chromosomal instability contributed fragility cytogeneticists molecular syndrome pathogenic regions aberrantly ideal transduction aberrant expression pathological susceptibility differentiation congenital breakage link unifying mechanisms located cell predisposition survival chemopreventive gene sites determined therein disease signal fanconi cfs marrow genes amplifications phenotypes models break model cellular mechanistic variations tumor mouse cancer originally dissect events anemia fa regulating pathogenesis cfss rearrangements bases copy deletions therapeutic

Project "FAtoUnFRAGILITY" data sheet

The following table provides information about the project.

Coordinator	CENTRE NATIONAL DE LA RECHERCHE SCIENTIFIQUE CNRS Organization address address: RUE MICHEL ANGE 3 city: PARIS postcode: 75794 website: www.cnrs.fr 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	France [FR]
Project website	https://www.gustaveroussy.fr/en/stress-replicatif-instabilite-genetique-et-mitose-thematique
Total cost	1˙462˙383 €
EC max contribution	1˙462˙383 € (100%)
Programme	1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
Code Call	ERC-2014-STG
Funding Scheme	ERC-STG
Starting year	2015
Duration (year-month-day)	from 2015-06-01 to 2020-05-31

Partnership

Take a look of project's partnership.

#	participants	country	role	EC contrib. [€]
1	CENTRE NATIONAL DE LA RECHERCHE SCIENTIFIQUE CNRS CENTRE NATIONAL DE LA RECHERCHE SCIENTIFIQUE CNRS Organization address address: RUE MICHEL ANGE 3 city: PARIS postcode: 75794 website: www.cnrs.fr contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	FR (PARIS)	coordinator	1˙462˙383.00

Map

Project objective

Originally described by cytogeneticists, common fragile sites (CFSs) are chromosomal regions known for their susceptibility to break and rearrange aberrantly, thus altering the expression of genes located therein. CFS instability is associated with tumor development and pathogenic copy number variations. Recent advances have significantly contributed to dissect the molecular bases of CFS instability, yet a unifying model for their unique breakage propensity has not been determined. Fanconi anemia (FA) is a chromosomal instability syndrome featuring congenital abnormalities, bone marrow failure and cancer predisposition, characterized by an increased CFS fragility. FA is thus an ideal model to understand the mechanisms underpinning CFS instability and the mechanistic link between CFS instability and the pathogenesis of disease phenotypes. I propose to use FA cellular models to examine the molecular events leading to CFS instability, and FA mouse models to investigate the consequences of deletions, amplifications or rearrangements involving CFSs on the expression of genes regulating critical signal transduction pathways involved in cell survival, proliferation, and differentiation. Exploring these mechanisms can lead to the development of chemopreventive or therapeutic strategies targeting aberrant gene expression or pathological pathways.

Publications

List of publications.
year	authors and title	journal	last update
2019	Philippe Fernandes, Benoit Miotto, Claude Saint-Ruf, Viola NÃ¤hse, Silvia Ravera, Enrico Cappelli, Valeria Naim FANCD2 tunes the UPR preventing mitochondrial stress--induced common fragile site instability published pages: , ISSN: , DOI: 10.1101/808915		2019-12-16
2019	Michalis Fragkos, Viviana Barra, Tom Egger, Benoit Bordignon, Delphine Lemacon, Valeria Naim and Arnaud Coquelle Dicer prevents genome instability in response to replication stress published pages: , ISSN: 1949-2553, DOI: 10.18632/oncotarget.27034	Oncotarget 10/43	2019-12-16
2017	Michalis Fragkos, Valeria Naim Rescue from replication stress during mitosis published pages: 1-21, ISSN: 1538-4101, DOI: 10.1080/15384101.2017.1288322	Cell Cycle	2019-05-29

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