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.

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

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.)

Widow Spider Mating (2020)

Immature mating as a novel tactic of an invasive widow spider

Read More  

LieLowerBounds (2019)

Lower bounds for partial differential operators on compact Lie groups

Read More  

TARGET SLEEP (2020)

Boosting motor learning through sleep and targeted memory reactivation in ageing and Parkinson’s disease

Read More