EUCLYD

A European Consortium for Lysosomal Disorders

Coordinatore	UNIVERSITA DEGLI STUDI DI NAPOLI FEDERICO II.    more  Organization address address: Corso Umberto I 40 city: NAPOLI postcode: 80138 contact info Titolo: Ms. Nome: Gaetana Cognome: Errico Email: send email Telefono: +39 081 7463365 Fax: +39 081 7463116
Nazionalità Coordinatore	Italy [IT]
Sito del progetto	http://www.euclyd.eu
Totale costo	3˙907˙480 €
EC contributo	2˙958˙310 €
Programma	FP7-HEALTH Specific Programme "Cooperation": Health
Code Call	FP7-HEALTH-2007-A
Funding Scheme	CP-FP
Anno di inizio	2008
Periodo (anno-mese-giorno)	2008-05-01   -   2011-04-30

 Partecipanti

#	participant	country	role	EC contrib. [€]
1	UNIVERSITA DEGLI STUDI DI NAPOLI FEDERICO II.  Organization address address: Corso Umberto I 40 city: NAPOLI postcode: 80138 contact info Titolo: Ms. Nome: Gaetana Cognome: Errico Email: send email Telefono: +39 081 7463365 Fax: +39 081 7463116	IT (NAPOLI)	coordinator	0.00
2	ERASMUS UNIVERSITAIR MEDISCH CENTRUM ROTTERDAM  Organization address address: 's Gravendijkwal 230 city: ROTTERDAM postcode: 3015CE contact info Titolo: Ms. Nome: Maria Aj Cognome: Van Zeijl Email: send email Telefono: -4087132 Fax: + 31 10 70 44 736	NL (ROTTERDAM)	participant	0.00
3	FONDAZIONE TELETHON  Organization address address: VIA VARESE 16/B city: ROMA postcode: 185 contact info Titolo: Ms. Nome: Irene Cognome: Mearelli Email: send email Telefono: +39 0644015308 Fax: +39 0644119224	IT (ROMA)	participant	0.00
4	LUNDS UNIVERSITET  Organization address address: Paradisgatan 5c city: LUND postcode: 22100 contact info Titolo: Mr. Nome: Jens Cognome: Forsberg Email: send email Telefono: -2220575 Fax: -2224550	SE (LUND)	participant	0.00
5	THE CHANCELLOR, MASTERS AND SCHOLARS OF THE UNIVERSITY OF CAMBRIDGE  Organization address address: The Old Schools, Trinity Lane city: CAMBRIDGE postcode: CB2 1TN contact info Titolo: Ms. Nome: Dawn Cognome: Barker Email: send email Telefono: -766336 Fax: -334167	UK (CAMBRIDGE)	participant	0.00
6	UNIVERSITATSMEDIZIN DER JOHANNES GUTENBERG-UNIVERSITAT MAINZ  Organization address address: Langenbeckstrasse 1 city: Mainz postcode: 55131 contact info Titolo: Ms. Nome: Sibel Cognome: Koctürk Email: send email Telefono: +49 6131175754 Fax: +49 6131175672	DE (Mainz)	participant	0.00

Mappa

 Word cloud

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 Obiettivo del progetto (Objective)

'The EUCLYD consortium aims at developing a scientific network among outstanding communities of basic and clinical investigators in five European countries (Italy, Netherlands, UK, Sweden and Germany) to study various aspects of lysosomal storage diseases (LSD). LSDs are rare disorders (overall prevalence of 1/5000 to 1/8000), each due to a specific lysosomal enzyme deficiency, leading to intracellular storage of a variety of undegraded substrates (sphingolipids, glycosaminoglycans, glycoproteins and glycogen) in different tissues. Among the 40 to 50 LSDs presently known, EUCLYD will focus on Gaucher disease, Pompe disease, MPS VI and Multiple sulfatase deficiency, as prototypes of disorders with different stored materials in various organs and tissues outside the CNS. The issues to be investigated in the proposed project are: pathophysiology and mechanisms underlying the symptoms and leading to devastating clinical consequences; natural history; testing of novel therapeutic approaches. These issues will be addressed by patients studies and with mouse models recapitulating the phenotype of LSDs. Recent advances in the pathophysiology of LSDs point to the role of secondary processes triggered by substrate storage. A better understanding of these processes is crucial to identify new targets of therapy. Enzyme replacement and substrate reduction therapies were successfully introduced in the treatment of specific LSDs. However, pilot clinical trials should be supported by post-marketing studies to further assess the efficacy of these therapies and to develop standardized treatment protocols. To this purpose a precise knowledge of the natural history of LSDs is essential. Novel therapeutic strategies, such as enzyme enhancement and gene therapy are still under investigation in cell systems and in animal models. EUCLYD will provide further testing of these approaches in order to translate state of the art knowledge into clinical applications and reach patient's bedside.'

Introduzione (Teaser)

Under normal physiological conditions, most of the body's cellular clearing processes occur in a specialised organelle, the lysosome. Impairment of lysosomal function leads to a variety of pathologies, usually associated with neurodegeneration.