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TAMYOCAL SIGNED

Tamoxifen mediated protection on X-linked centronuclear myopathy: a mechanistic and pre-clinical study

Total Cost €

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EC-Contrib. €

0

Partnership

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 TAMYOCAL project word cloud

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

leg       restricting    instrumental    mtorc1    drugs    partly    igf    complete    options    missing    mechanism    idea    protects    skeletal    steroids    estrogen    rationale    shown    action    acts    families    group    multiple    hosting    extends    approved    mice    autophagy    diseases    function    premature    determined    wild    first    treatment    unbalanced    survival    vivo    clinical    atrophy    caused    molecular    estrogenic    mimics    modulator    pilot    explained    combined    modulating    lifespan    elucidating    tamoxifen    births    treated    everolimus    enhancement    death    rare    signalling    creatine    therapeutic    myopathies    selective    symptoms    model    players    dramatic    xlcnm    severe    mouse    rate    balance    lipid    rescue    absence    myotubularin    treatments    centronuclear    functional    efficacy    parallel    congenital    patients    phosphoinositide    events    myopathy    male    exact    prevents    compounds    tam    muscle    phosphatases    phosphatase    receptor    positively    linked   

Project "TAMYOCAL" data sheet

The following table provides information about the project.

Coordinator
UNIVERSITE DE GENEVE 

Organization address
address: RUE DU GENERAL DUFOUR 24
city: GENEVE
postcode: 1211
website: www.unige.ch

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 Switzerland [CH]
 Total cost 175˙419 €
 EC max contribution 175˙419 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2017
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2018
 Duration (year-month-day) from 2018-05-01   to  2020-04-30

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    UNIVERSITE DE GENEVE CH (GENEVE) coordinator 175˙419.00

Map

 Project objective

X-linked centronuclear myopathy (XLCNM) is a severe congenital myopathy caused by the absence of lipid phosphatase myotubularin. XLCNM affects 1/50.000 male births, there is no treatment and many cases lead to premature death. Thus, treatments are needed. Tamoxifen (TAM) is a selective estrogen receptor modulator that mimics estrogen signalling in skeletal muscle. Pilot study and previous results from the hosting group, have shown that TAM improve muscle symptoms and survival rate in XLCNM mouse model. Results strongly support the idea that TAM protects skeletal muscle via enhancement of estrogenic signalling positively modulating multiple pathways linked to muscle function. However, the exact mechanism(s) is not yet understood. This project aims at elucidating the mechanism(s) of action of TAM and evaluate further its efficacy in XLCNM mouse model. Two specific aims are proposed. First, to determine how TAM acts on pathways and key players involved in XLCNM. Estrogen signalling, phosphoinositide balance, autophagy and mTORC1 signalling will be determined in wild type and XLCNM mice treated and non-treated by TAM. Second, to investigate the pre-clinical efficacy of TAM combined with other compounds in XLCNM mice. TAM extends the lifespan of XLCNM mice according to the pilot study but it prevents only partly leg muscle atrophy, restricting complete functional rescue. Thus, TAM will be combined with other approved drugs that target muscle atrophy (everolimus, creatine, IGF-I and steroids). These parallel approaches will provide knowledge about not-yet explained events related to XLCNM and TAM effects, from molecular to in-vivo level, and instrumental information for other myopathies and rare diseases where lipid phosphatases are missing and phosphoinositide are unbalanced. This knowledge might provide the rationale for new therapeutic options for these dramatic conditions, being highly relevant to patients and families.

 Publications

year authors and title journal last update
List of publications.
2018 Elinam Gayi, Laurence A. Neff, Xènia Massana Muñoz, Hesham M. Ismail, Marta Sierra, Thomas Mercier, Laurent A. Décosterd, Jocelyn Laporte, Belinda S. Cowling, Olivier M. Dorchies, Leonardo Scapozza
Tamoxifen prolongs survival and alleviates symptoms in mice with fatal X-linked myotubular myopathy
published pages: , ISSN: 2041-1723, DOI: 10.1038/s41467-018-07058-4
Nature Communications 9/1 2019-05-18

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