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NeuroLSD SIGNED

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

Total Cost €

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EC-Contrib. €

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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.

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

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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