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BONEPHAGY SIGNED

Defining the role of the FGF – autophagy axis in bone physiology

Total Cost €

EC-Contrib. €

Partnership

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BONEPHAGY project word cloud

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

pathological secretion context cellular explore fgfr3 pharmacological links cell regulation regulate critical intracellular physiological physio mouse bone materials signalling fgf combines regulator achondroplasia hence data cartilage disorders recycling signaling play deputed opportunity dwarfisms modulation variety induces played human cells treat ach skeletal cartilaginous mice machinery mechanisms deregulated chondrocyte diseases mutations catabolic therapeutic light dysplasia postnatal degradation impairment roles preliminary discovered pathogenesis homeostasis extracellular grant plate autophagy maintenance ligands biology relevance genetics organismal molecular thanathoporic collagen fgf18 shed caused multiple fundamental matrix plays showing models treatment uncover receptors

Project "BONEPHAGY" data sheet

The following table provides information about the project.

Coordinator	FONDAZIONE TELETHON Organization address address: VIA VARESE 16/B city: ROMA postcode: 185 website: www.telethon.it 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	Italy [IT]
Total cost	1˙586˙430 €
EC max contribution	1˙586˙430 € (100%)
Programme	1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
Code Call	ERC-2016-STG
Funding Scheme	ERC-STG
Starting year	2017
Duration (year-month-day)	from 2017-01-01 to 2021-12-31

Partnership

Take a look of project's partnership.

#	participants	country	role	EC contrib. [€]
1	FONDAZIONE TELETHON FONDAZIONE TELETHON Organization address address: VIA VARESE 16/B city: ROMA postcode: 185 website: www.telethon.it contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	IT (ROMA)	coordinator	1˙586˙430.00

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Project objective

Autophagy is a fundamental cellular catabolic process deputed to the degradation and recycling of a variety of intracellular materials. Autophagy plays a significant role in multiple human physio-pathological processes and is now emerging as a critical regulator of skeletal development and homeostasis. We have discovered that during postnatal development in mice, the growth factor FGF18 induces autophagy in the chondrocyte cells of the growth plate to regulate the secretion of type II collagen, a major component of cartilaginous extracellular matrix. The FGF signaling pathways play crucial roles during skeletal development and maintenance and are deregulated in many skeletal disorders. Hence our findings may offer the unique opportunity to uncover new molecular mechanisms through which FGF pathways regulate skeletal development and maintenance and to identify new targets for the treatment of FGF-related skeletal disorders. In this grant application we propose to study the role played by the different FGF ligands and receptors on autophagy regulation and to investigate the physiological relevance of these findings in the context of skeletal growth, homeostasis and maintenance. We will also investigate the intracellular machinery that links FGF signalling pathways to the regulation of autophagy. In addition, we generated preliminary data showing an impairment of autophagy in chondrocyte models of Achondroplasia (ACH) and Thanathoporic dysplasia, two skeletal disorders caused by mutations in FGFR3. We propose to study the role of autophagy in the pathogenesis of FGFR3-related dwarfisms and explore the pharmacological modulation of autophagy as new therapeutic approach for achondroplasia. This application, which combines cell biology, mouse genetics and pharmacological approaches, has the potential to shed light on new mechanisms involved in organismal development and homeostasis, which could be targeted to treat bone and cartilage diseases.

Publications

List of publications.
year	authors and title	journal	last update
2017	Rosa Bartolomeo, Laura Cinque, Chiara De Leonibus, Alison Forrester, Anna Chiara Salzano, Jlenia Monfregola, Emanuela De Gennaro, Edoardo Nusco, Isabella Azario, Carmela Lanzara, Marta Serafini, Beth Levine, Andrea Ballabio, Carmine Settembre mTORC1 hyperactivation arrests bone growth in lysosomal storage disorders by suppressing autophagy published pages: 3717-3729, ISSN: 0021-9738, DOI: 10.1172/JCI94130	Journal of Clinical Investigation 127/10	2019-04-03

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