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Neurovulnerability SIGNED

Molecular mechanisms underlying selective neuronal death in motor neuron diseases

Total Cost €

0

EC-Contrib. €

0

Partnership

0

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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.

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

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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