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MitSyn

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

Total Cost €

0

EC-Contrib. €

0

Partnership

0

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 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.

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

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

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