EUCLYD

A European Consortium for Lysosomal Disorders

 Coordinatore 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

 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

Esplora la "nuvola delle parole (Word Cloud) per avere un'idea di massima del progetto.

clinical    tissues    history    lsds    substrate    storage    therapeutic    models    deficiency    euclyd    therapies    natural    enzyme    pathophysiology    treatment    variety    disorders    lysosomal    therapy    disease   

 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.

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