Opendata, web and dolomites

MitSyn

Alpha-synuclein and mitochondrial dysfunction: key links between Gaucher’s disease and Parkinson’s?

Total Cost €

0

EC-Contrib. €

0

Partnership

0

Views

0

 MitSyn project word cloud

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

reduce    models    techniques    mitochondrial    defects    glucocerebrosidase    disorder    model    accumulation    alpha    array    autophagy    insights    lewy    proteins    storage    mouse    relate    imaging    children    primary    highlighted    enzyme    clearance    pathophysiology    lysosomal    association    bodies    hypothesis    parkinson    mutations    oligomerization    shares    pathophysiological    causing    generate    silenced    fact    protein    causes    function    molecular    gcase    recent    disease    interactions    shown    knockout    mice    diverse    pd    neurodegeneration    signaling    inducible    overexpressed    gaucher    therapeutic    genetic    attempt    patients    cells    synuclein    pluripotent    shows    gd    cultures    biochemical    forms    dys    severe    inclusions    biophysical    unclear    brains    quality    unifying    mechanisms    parkinsonian    aggregates    give    impaired    sporadic    brings    neuronal    intracellular    cellular    gba    oligomeric    dysfunction    found    quantitatively    constituent    stem   

Project "MitSyn" data sheet

The following table provides information about the project.

Coordinator
UNIVERSITY COLLEGE LONDON 

Organization address
address: GOWER STREET
city: LONDON
postcode: WC1E 6BT
website: n.a.

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 United Kingdom [UK]
 Total cost 183˙454 €
 EC max contribution 183˙454 € (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 2015-04-13   to  2017-04-12

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    UNIVERSITY COLLEGE LONDON UK (LONDON) coordinator 183˙454.00

Map

 Project objective

Recent studies have highlighted a strong genetic association between Parkinson’s disease (PD) and Gaucher’s disease (GD), a lysosomal storage disorder that causes severe neurodegeneration in children and shares some pathophysiological features of PD. GD results from mutations that reduce activity of the enzyme glucocerebrosidase (GCase), causing impaired lysosomal function. It was recently shown that GCase mutations cause several defects associated with impaired cellular quality control - impaired autophagy, mitochondrial dysfunction and accumulation of oligomeric alpha-synuclein, a protein strongly involved in sporadic and genetic PD forms. In fact, alpha-synuclein aggregates are the main constituent of Lewy Bodies, characteristic proteins inclusions found in parkinsonian brains. While PD shows similar features and involves defects in the same pathways, it remains unclear how these diverse findings relate to each other. In the present study we propose to identify the specific interactions between intracellular signaling pathways, cellular quality control pathways and alpha-synuclein oligomerization in an attempt to generate a unifying hypothesis that brings together known features of GD and PD pathophysiology. We will use primary neuronal cultures from gba knockout mice and inducible pluripotent stem cells derived from the GD mouse model and from patients, in which alpha-synuclein will be overexpressed or silenced. Mitochondrial (dys)function, impaired clearance mechanisms and alpha-synuclein oligomerization will be quantitatively characterized in these models by means of an array of biochemical, biophysical and advanced imaging techniques. The results of the work will give us further insights into PD molecular mechanisms and may provide new therapeutic targets.

 Publications

year authors and title journal last update
List of publications.
2017 Nicoletta Plotegher, Michael R. Duchen
Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders
published pages: 116-134, ISSN: 1471-4914, DOI: 10.1016/j.molmed.2016.12.003
Trends in Molecular Medicine 23/2 2019-07-23

Are you the coordinator (or a participant) of this project? Plaese send me more information about the "MITSYN" 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 "MITSYN" are provided by the European Opendata Portal: CORDIS opendata.

More projects from the same programme (H2020-EU.1.3.2.)

GW (2019)

Analysing the heavy element factories of the Universe : photometric and spectroscopic sample study of kilonovae

Read More  

TRACE-AD (2019)

Tracking the Effects of Amyloid and Tau Pathology on Brain Systems and Cognition in Early Alzheimer’s Disease

Read More  

ICARUS (2020)

Information Content of locAlisation: fRom classical to qUantum Systems

Read More