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RESEARCH GROUPS
Group U759
Programme: Inherited Metabolic Medicine
Lead Researcher: Pujol Onofre, Aurora Group Members
STAFF MEMBERS: Launay, Nathalie | Ruiz Sales, Montserrat
ASSOCIATED MEMBERS: Coppa, Andrea | Dalfo Capella, Esther | Fourcade Guillou, Stephane | Grau Gui- jarro, Laia | Guilera Zapater, Cristina | Martínez García, Juan José | Ranea Robles, Pablo | Schluter Martín, Agatha
Main lines of research
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• • • • •
Physiopathology of adrenoleukodystrophy: impact of oxidative stress in mitochondrial function, ener- getic homeostasis and proteolytic processes, using the mouse model developed and characterized in our laboratory and tissues of X-ALD patients .
Treatment of adrenoleukodystrophy: preclinical tests in the mouse model and clinical trials in patients with X-ALD .
Peroxisomal integrative genomics . Peroxisomal metabolome characterization and the organelle’s evo- lutive origin .
Physiopathology of Pelizaeus Merzbacher disease, metachromatic leukodystrophy and other leuko- dystrophies .
Disease model of adrenoleukodystrophy in C .elegans: role of fatty acids in oxidative stress, neurode- generation and aging .
Systems biomedicine for unravelling the molecular basis and modelling leukodystrophies and inheri- ted spastic paraplegias .
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