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.

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

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.)

LO-KMOF (2019)

Vapour-deposited metal-organic frameworks as high-performance gap-filling dielectrics for nanoelectronics

Read More  

PLAT_ACE (2019)

A new platform technology for the on-demand access to large acenes

Read More  

TRUST (2018)

Truth and Semantics

Read More