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NMJALS SIGNED

In vivo analysis of neuromuscular junction stability in zebrafish models of amyotrophic lateral sclerosis

Total Cost €

0

EC-Contrib. €

0

Partnership

0

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 NMJALS project word cloud

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

contexts    constituting    characterise    patient    fish    characterisation    maintenance    quantification    stability    lethal    neurodegenerative    plan    genetic    neuromuscular    junctions    lateral    specificity    proteins    patients    quantitative    innovative    subtle    degeneration    precise    amyotrophic    neuronal    starts    protein    neuron    tools    super    identification    live    relevance    cell    vertebrate    destabilisation    stabilisation    fluorescent    detecting    molecular    model    multidisciplinary    biology    organisation    mutations    sclerosis    molecules    technically    microscopy    death    therapeutic    genes    disease    adhesion    unravel    appear    clock    counteract    dynamic    resolution    motor    defects    individuals    analysing    excellent    interesting    onset    imaging    event    zebrafish    computational    turnover    neurons    als    therapies    point    progression    dynamics    unrelated    regulatory    mechanisms    causes    largely    cytoskeleton    combine    nmj    equilibrium    regardless    slow    seem    modifications    understand    preceding   

Project "NMJALS" data sheet

The following table provides information about the project.

Coordinator
INSTITUT CURIE 

Organization address
address: rue d'Ulm 26
city: PARIS
postcode: 75231
website: www.curie.fr

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 France [FR]
 Total cost 185˙076 €
 EC max contribution 185˙076 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2014
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2015
 Duration (year-month-day) from 2015-11-16   to  2017-11-15

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    INSTITUT CURIE FR (PARIS) coordinator 185˙076.00

Map

 Project objective

Amyotrophic lateral sclerosis (ALS) is a late onset, lethal neurodegenerative disease of motor neurons that affects about 2 in 100,000 individuals per year. Different genetic mutations have been described in patients, but the genes involved seem unrelated and the causes of the disease appear complex and are still largely not understood. Of relevance, the destabilisation of neuromuscular junctions (NMJ) may be an early event, preceding neuronal death, making them an interesting therapeutic target to slow down disease progression. The goal of the study is to characterise the molecular organisation and dynamics of NMJ, in particular of cell adhesion molecules, in order to understand the mechanisms of maintenance of NMJ by analysing defects in their organisation in ALS contexts. Technically, we plan to combine neuron biology, quantitative cell biology and computational tools to reach a precise molecular understanding of the dynamic equilibrium of NMJ in zebrafish, an excellent vertebrate model for live imaging. We will develop innovative methods of super-resolution microscopy and of quantification of protein turnover using a fluorescent molecular clock. This precise molecular characterisation of the NMJ will allow detecting subtle early changes in the NMJ that occur before neuronal degeneration starts, in fish carrying mutations described in ALS patients. The project will lead to the identification of key proteins for NMJ stabilisation and enable the analysis of regulatory elements of this stability, especially the cytoskeleton. The innovative multidisciplinary approach of this study will unravel early modifications in the organisation of the NMJ preceding neuronal degeneration in ALS contexts. It will point out key proteins of NMJ maintenance constituting new targets for the development of therapies to counteract destabilisation of neuromuscular junctions, regardless of the genetic specificity of the patient.

 Publications

year authors and title journal last update
List of publications.
2017 Shahad Albadri, Filippo Del Bene, Céline Revenu
Genome editing using CRISPR/Cas9-based knock-in approaches in zebrafish
published pages: 77-85, ISSN: 1046-2023, DOI: 10.1016/j.ymeth.2017.03.005
Methods 121-122 2019-06-18

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