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NeuroLSD SIGNED

Neuro-metabolic, structural and functional hallmarks of Lysosomal Storage Diseases

Total Cost €

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EC-Contrib. €

0

Partnership

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 NeuroLSD project word cloud

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

spin    severe    reliably    cutting    clinical    vienna    accelerated    diffusion    pseudo    lsd    emerges    resonance    neurodegenerative    deficits    medical    alzheimer    methodology    critically    enlarged    technique    storage    blood    morphological    edge    accumulation    chaperones    t2    fabry    weighted    trials    gaucher    relevance    malfunctioning    diseases    markers    brain    sensitively    administration    pathology    intracellular    reproducible    bypass    mucopolysaccharidosis    metabolites    alteration    metabolic    continuous    excessive    treatments    central    cognitive    progressive    mr    continuing    share    organ    utilize    brains    enzyme    magnetic    psychological    combination    perivascular    levels    triggers    nervous    lysosomal    age    optimal    boost    damage    t1    extents    mps    barrier    proton    vivo    glycocerebroside    intrathecal    track    comprehensively    functional    leukodystrophy    mri    disease    globotriaosylceramide    parkinson    gd    overcome    resolution    spaces    gene    labeling    spectroscopic    arterial    imaging    abnormalities    substances    ratio    describe    prognostic    glycosaminoglycans    quantify    therapies    search    patients    ranging    protocol    atrophy    university    microstructural   

Project "NeuroLSD" data sheet

The following table provides information about the project.

Coordinator
MEDIZINISCHE UNIVERSITAET WIEN 

Organization address
address: SPITALGASSE 23
city: WIEN
postcode: 1090
website: www.meduniwien.ac.at

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 Austria [AT]
 Total cost 166˙156 €
 EC max contribution 166˙156 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2017
 Funding Scheme MSCA-IF-EF-RI
 Starting year 2018
 Duration (year-month-day) from 2018-10-01   to  2021-07-18

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    MEDIZINISCHE UNIVERSITAET WIEN AT (WIEN) coordinator 166˙156.00

Map

 Project objective

Continuing development of novel brain treatments, which bypass blood-brain barrier, further emerges the need to establish prognostic magnetic resonance (MR) markers to track progressive brain alteration in lysosomal storage diseases (LSD). Excessive intracellular accumulation of lysosomal substances such as glycosaminoglycans in mucopolysaccharidosis (MPS), globotriaosylceramide in Fabry disease and glycocerebroside in Gaucher disease (GD) triggers multi-organ malfunctioning and significant damage to the central nervous system. While LSD are associated with various levels of cognitive deficits, and distinct extents of morphological brain abnormalities (e.g., atrophy, leukodystrophy or enlarged perivascular spaces) ranging from non-existing in GD type 1 to severe in MPS type 2, microstructural and metabolic processes have not been comprehensively described in brains of LSD patients in vivo yet. We will utilize cutting-edge accelerated proton MR spectroscopic imaging methodology that was developed at the Medical University of Vienna in combination with advanced diffusion MRI, high-resolution T1-/T2-weighted ratio, and pseudo-continuous arterial spin labeling technique. Our protocol will reliably quantify levels of all relevant brain metabolites, while sensitively describe microstructural and functional deficits to determine the relevance of MR measures in psychological deficits in LSD. Reproducible MR methods are needed to assess effects of novel treatments that overcome blood-brain barrier such as intrathecal enzyme administration, chaperones and gene therapies in clinical LSD trials. Increased understanding of brain LSD pathology will critically boost search of optimal therapies in age-related neurodegenerative diseases that share some common features with LSD such as Alzheimer’s or Parkinson’s disease.

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The information about "NEUROLSD" are provided by the European Opendata Portal: CORDIS opendata.

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