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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

0

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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.

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

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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