Opendata, web and dolomites

Neurovulnerability SIGNED

Molecular mechanisms underlying selective neuronal death in motor neuron diseases

Total Cost €

0

EC-Contrib. €

0

Partnership

0

Views

0

 Neurovulnerability project word cloud

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

axonal    functions    therapeutics    directions    human    sclerosis    assembly    postmitotic    conserved    drosophila    fascinating    crispr    dysfunction    name    lysosome    neurodegenerative    amyotrophic    lateral    devastating    neuron    degeneration    basis    accumulation    patient    cell    deficiency    altogether    generate    phases    mechanisms    spliceosome    atrophy    als    engineer    underlying    neurodegeneration    subtype    receives    damaged    neuronal    spinal    cas9    expressed    clues    intracellular    smn    survival    selective    lines    isogenic    types    follow    vulnerability    single    proteostatic    mrna    protein    untangling    mutations    hallmark    neurons    nature    ipscs    deficit    subtypes    molecular    transport    probability    hypotheses    question    ubiquitously    levels    muscular    death    populations    catabolic    aggregates    genetically    autophagy    organelles    mn    leads    heterogeneity    interdisciplinary    motor    mnds    reporter    quality    mnd    diseases    genes    specialized    sma    model    critical    neuropathology    recycling    unknown    hypothesis   

Project "Neurovulnerability" data sheet

The following table provides information about the project.

Coordinator
DEUTSCHES ZENTRUM FUR NEURODEGENERATIVE ERKRANKUNGEN EV 

Organization address
address: SIGMUND FREUD STRASSE 27
city: BONN
postcode: 53127
website: www.dzne.de

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 Germany [DE]
 Total cost 1˙472˙667 €
 EC max contribution 1˙472˙667 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2018-STG
 Funding Scheme ERC-STG
 Starting year 2019
 Duration (year-month-day) from 2019-08-01   to  2024-07-31

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    DEUTSCHES ZENTRUM FUR NEURODEGENERATIVE ERKRANKUNGEN EV DE (BONN) coordinator 1˙472˙667.00
2    AGENCIA ESTATAL CONSEJO SUPERIOR DEINVESTIGACIONES CIENTIFICAS ES (MADRID) participant 0.00

Map

 Project objective

The mechanisms behind neuronal death in different motor neuron diseases (MND) remain unknown. These MNDs include the devastating spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS). A fascinating question in neurodegeneration research is why mutations in ubiquitously expressed genes result in the selective death of a specific neuronal subtype. The ubiquitously expressed and conserved survival of motor neuron (SMN) protein receives its name because its deficit results in MN degeneration. However, SMN known functions -spliceosome assembly and axonal mRNA transport- do not explain the selective MN vulnerability. Accumulation of intracellular aggregates in neurons is a hallmark of most neurodegenerative diseases. The lysosome-autophagy system is the main catabolic pathway for recycling of protein aggregates and damaged organelles, and its role as a quality control system is especially critical in neurons, due to their postmitotic and highly specialized nature. The hypothesis for this proposal is that SMN deficiency leads to a lysosome-autophagy dysfunction which results in a proteostatic failure, underlying MN degeneration. Furthermore, the existing heterogeneity in SMN protein levels across MN populations may determine their probability of survival. To test these hypotheses we will use the CRISPR/Cas9 system to genetically engineer human control, SMA and ALS patient-derived iPSCs to generate isogenic and reporter lines that will allow us to study selective neuronal subtypes at a single-cell level. We will also follow an interdisciplinary approach using a SMA Drosophila model to identify new molecular pathways essential for SMN neuropathology. Altogether, my research proposal aims at untangling the molecular mechanisms underlying selective MN death. Our results will open up new directions of research into the molecular basis of neurodegeneration and will provide clues for the design of therapeutics targeting specific neuronal types or phases of MNDs.

Are you the coordinator (or a participant) of this project? Plaese send me more information about the "NEUROVULNERABILITY" 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 "NEUROVULNERABILITY" are provided by the European Opendata Portal: CORDIS opendata.

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

REPLAY_DMN (2019)

A theory of global memory systems

Read More  

HYDROGEN (2019)

HighlY performing proton exchange membrane water electrolysers with reinforceD membRanes fOr efficient hydrogen GENeration

Read More  

E-DIRECT (2020)

Evolution of Direct Reciprocity in Complex Environments

Read More