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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.

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

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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