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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.

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

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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