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Neurovulnerability SIGNED

Molecular mechanisms underlying selective neuronal death in motor neuron diseases

Total Cost €

0

EC-Contrib. €

0

Partnership

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 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.

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

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.

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The information about "NEUROVULNERABILITY" are provided by the European Opendata Portal: CORDIS opendata.

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