Opendata, web and dolomites

ALS-Networks SIGNED

Defining functional networks of genetic causes for ALS and related neurodegenerative disorders

Total Cost €

0

EC-Contrib. €

0

Partnership

0

Views

0

 ALS-Networks project word cloud

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

fundamental    samples    first    lines    autophagy    death    patient    sqstm1    health    prevalent    molecular    innovative    amenable    disease    representing    proposing    variants    alleles    expand    mechanisms    vertebrate    rna    motor    population    pathological    interact    strategies    effect    pathophysiological    underlie    degradation    combine    regulation    outcome    genetic    validating    neuron    interactors    modifiers    cord    fatal    causative    neurodegeneration    tdp    relevance    expression    model    transgenic    content    neuroprotective    models    pharmacological    function    spinal    interaction    pathogenic    therapeutic    groundbreaking    als    indicates    pinpoint    tools    simultaneous    permit    diseases    stress    billion    800    animal    neurodegenerative    caused    causing    knockout    screened    power    bioactive    functional    genomic    brain    amongst    contributed    extend    multiple    editing    network    compounds    discovery    phenotype    gene    demonstrated    fus    38    neurological    markers    disorders    c9orf72    invariably    screens    interactions    cascades    granule    43    zebrafish    functionally    annually    mutations    cellular    converging    mutant    genes    uncover    genetically   

Project "ALS-Networks" data sheet

The following table provides information about the project.

Coordinator
INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE 

Organization address
address: RUE DE TOLBIAC 101
city: PARIS
postcode: 75654
website: www.inserm.fr

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 France [FR]
 Total cost 2˙000˙000 €
 EC max contribution 2˙000˙000 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2015-CoG
 Funding Scheme ERC-COG
 Starting year 2017
 Duration (year-month-day) from 2017-04-01   to  2022-03-31

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE FR (PARIS) coordinator 2˙000˙000.00

Map

 Project objective

Brain and spinal cord diseases affect 38% of the European population and cost over 800 billion € annually; representing by far the largest health challenge. ALS is a prevalent neurological disease caused by motor neuron death with an invariably fatal outcome. I contributed to ALS research with the groundbreaking discovery of TDP-43 mutations, functionally characterized these mutations in the first vertebrate model and demonstrated a genetic interaction with another major ALS gene FUS. Emerging evidence indicates that four major causative factors in ALS, C9orf72, TDP-43, FUS & SQSTM1, genetically interact and could function in common cellular mechanisms. Here, I will develop zebrafish transgenic lines for all four genes, using state of the art genomic editing tools to combine simultaneous gene knockout and expression of the mutant alleles. Using these innovative disease models I will study the functional interactions amongst these four genes and their converging effect on key ALS pathogenic mechanisms: autophagy degradation, stress granule formation and RNA regulation. These studies will permit to pinpoint the molecular cascades that underlie ALS-related neurodegeneration. We will further expand the current ALS network by proposing and validating novel genetic interactors, which will be further screened for disease-causing variants and as pathological markers in patient samples. The power of zebrafish as a vertebrate model amenable to high-content phenotype-based screens will enable discovery of bioactive compounds that are neuroprotective in multiple animal models of disease. This project will increase the fundamental understanding of the relevance of C9orf72, TDP-43, FUS and SQSTM1 by developing animal models to characterize common pathophysiological mechanisms. Furthermore, I will uncover novel genetic, disease-related and pharmacological modifiers to extend the ALS network that will facilitate development of therapeutic strategies for neurodegenerative disorders

 Publications

year authors and title journal last update
List of publications.
2018 Hortense de Calbiac, Adriana Dabacan, Elise Marsan, Hervé Tostivint, Gabrielle Devienne, Saeko Ishida, Eric Leguern, Stéphanie Baulac, Raul C. Muresan, Edor Kabashi, Sorana Ciura
Depdc5 knockdown causes mTOR-dependent motor hyperactivity in zebrafish
published pages: 510-523, ISSN: 2328-9503, DOI: 10.1002/acn3.542
Annals of Clinical and Translational Neurology 5/5 2019-05-14
2017 Shunmoogum A. Patten, Dina Aggad, Jose Martinez, Elsa Tremblay, Janet Petrillo, Gary A.B. Armstrong, Alexandre La Fontaine, Claudia Maios, Meijiang Liao, Sorana Ciura, Xiao-Yan Wen, Victor Rafuse, Justin Ichida, Lorne Zinman, Jean-Pierre Julien, Edor Kabashi, Richard Robitaille, Lawrence Korngut, J. Alexander Parker, Pierre Drapeau
Neuroleptics as therapeutic compounds stabilizing neuromuscular transmission in amyotrophic lateral sclerosis
published pages: , ISSN: 2379-3708, DOI: 10.1172/jci.insight.97152
JCI Insight 2/22 2019-05-14

Are you the coordinator (or a participant) of this project? Plaese send me more information about the "ALS-NETWORKS" project.

For instance: the website url (it has not provided by EU-opendata yet), the logo, a more detailed description of the project (in plain text as a rtf file or a word file), some pictures (as picture files, not embedded into any word file), twitter account, linkedin page, etc.

Send me an  email (fabio@fabiodisconzi.com) and I put them in your project's page as son as possible.

Thanks. And then put a link of this page into your project's website.

The information about "ALS-NETWORKS" are provided by the European Opendata Portal: CORDIS opendata.

More projects from the same programme (H2020-EU.1.1.)

Aware (2019)

Aiding Antibiotic Development with Deep Analysis of Resistance Evolution

Read More  

Resonances (2019)

Resonances and Zeta Functions in Smooth Ergodic Theory and Geometry

Read More  

FunDiT (2019)

Functional Diversity of T cells

Read More