BIG-HEART

Bench-to-beside Integrated approach to familial hypertrophic cardiomyopathy: to the HEART of the disease

Coordinatore	THE CHANCELLOR, MASTERS AND SCHOLARS OF THE UNIVERSITY OF OXFORD    more  Organization address address: University Offices, Wellington Square city: OXFORD postcode: OX1 2JD contact info Titolo: Mr. Nome: Stephen Cognome: Conway Email: send email Telefono: +44 01865234653
Nazionalità Coordinatore	United Kingdom [UK]
Sito del progetto	http://www.big-heart.eu/
Totale costo	3˙768˙620 €
EC contributo	2˙912˙992 €
Programma	FP7-HEALTH Specific Programme "Cooperation": Health
Code Call	FP7-HEALTH-2009-single-stage
Funding Scheme	CP-FP
Anno di inizio	2010
Periodo (anno-mese-giorno)	2010-01-01   -   2013-06-30

 Partecipanti

#	participant	country	role	EC contrib. [€]
1	THE CHANCELLOR, MASTERS AND SCHOLARS OF THE UNIVERSITY OF OXFORD  Organization address address: University Offices, Wellington Square city: OXFORD postcode: OX1 2JD contact info Titolo: Mr. Nome: Stephen Cognome: Conway Email: send email Telefono: +44 01865234653	UK (OXFORD)	coordinator	728˙655.50
2	IMPERIAL COLLEGE OF SCIENCE, TECHNOLOGY AND MEDICINE  Organization address address: SOUTH KENSINGTON CAMPUS EXHIBITION ROAD city: LONDON postcode: SW7 2AZ contact info Titolo: Mr. Nome: Michael Cognome: Robinson Email: send email Telefono: +44 020 594 3866 Fax: +44 0207 594 3868	UK (LONDON)	participant	592˙968.60
3	UNIVERSITA DEGLI STUDI DI FIRENZE  Organization address address: Piazza San Marco 4 city: Florence postcode: 50121 contact info Titolo: Prof. Nome: Elisabetta Cognome: Cerbai Email: send email Telefono: +39 0554271264 Fax: +39 0554271280	IT (Florence)	participant	540˙153.00
4	UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF  Organization address address: Martinistrasse 52 city: HAMBURG postcode: 20246 contact info Titolo: Dr. Nome: Lucie Cognome: Carrier Email: send email Telefono: 4940740000000 Fax: 4940740000000	DE (HAMBURG)	participant	500˙707.66
5	VERENIGING VOOR CHRISTELIJK HOGER ONDERWIJS WETENSCHAPPELIJK ONDERZOEK EN PATIENTENZORG  Organization address address: De Boelelaan 1105 city: AMSTERDAM postcode: 1081 HV contact info Titolo: Mr. Nome: Remco Cognome: Immerzeel Email: send email Telefono: +31 20 44 42144 Fax: +31 20 44 44470	NL (AMSTERDAM)	participant	415˙056.80
6	CF CONSULTING FINANZIAMENTI UNIONE EUROPEA SRL  Organization address address: Via Giuseppe Mussi 1 city: MILANO postcode: 20154 contact info Titolo: Mrs. Nome: Carla Cognome: Finocchiaro Email: send email Telefono: 39231083310 Fax: 39233614064	IT (MILANO)	participant	135˙450.42
7	STICHTING VU-VUMC  Organization address address: DE BOELELAAN 1105 city: AMSTERDAM postcode: 1081 HV contact info Nome: Michel Cognome: Telkamp Email: send email Telefono: +31 20 444 8190	NL (AMSTERDAM)	participant	0.00

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 Obiettivo del progetto (Objective)

'Hypertrophic cardiomyopathy (HCM), characterised by left ventricular hypertrophy and myocyte disarray, is by far the most common cardiac single gene disorder. With a prevalence of 1:500, HCM is predicted to affect approximately one million people within the EU. HCM represents an important clinical problem, being the principal cause of sudden death in young adults, and a valuable opportunity to use human genetics to dissect mechanisms of cardiac hypertrophy and heart failure. It was the first inherited heart disease to be characterised at the molecular genetic level, with the demonstration that it is caused by mutations in genes that encode different components of the cardiac contractile apparatus. Existing in vitro and mouse model studies have suggested that HCM mutations enhance contractility and impair relaxation. We have hypothesised that these changes may result in energetic compromise, due to inefficient ATP utilisation, and also in altered Ca2 handling. If validated in the human heart, these hypotheses would identify tractable therapeutic targets that suggest that HCM, perhaps more than any other cardiomyopathy, will be amenable to disease modifying therapy. However, analysis of the altered functional characteristics of affected human tissue has so far lagged behind the in vitro and mouse studies. In this project, we aim: (i) to collect and genotype a large collection of both affected HCM and appropriate control human myocardium and to measure the differences in protein expression, contractility, protein phosphorylation and sarcomeric structure between normal and affected samples; (ii) to test therapeutic strategies in our existing mouse models; (iii) to employ patient-based studies to test specific interventions based on existing hypotheses. This will provide a broad multidisciplinary approach to gain further understanding HCM and to yield new directions for therapeutic strategies.'