Neurometabolic Diseases Lab

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Neurometabolic Diseases Lab

Our Lab

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The Neurometabolic Diseases Lab, led by ICREA Research Professor Aurora Pujol, is integrated in the Neurosciences Area of IDIBELL. The lab was created in January 2005 thanks to the support of the Catalan Institution for Research and Advanced Studies (ICREA), the European Commission (FP6 program) and the European Leukodystrophy Association.

Our main interest lies on deciphering the pathophysiology of neurometabolic diseases for developing rational therapeutic strategies. For this aim, we are using state-of-the-art-technology, including transcriptomics, proteomics, metabolomics and lipidomics following an integrative, systems biology approach. We have mostly focused our work on elucidating the molecular basis of X-adrenoleukodystrophy, in particular towards oxidative stress and mitochondrial dysfunction pathways. Further, we are using a comparative genomics approach to studying the evolutionary origin of peroxisomes and their related metabolic routes, with focus in lipid metabolism. The derived knowledge might contribute to unravelling the role of this poorly studied organelle in ageing and neurodegenerative diseases.

The Neurometabolic Diseases Lab is as today a consolidated research group recognised by the Catalan Government (GRC 85). The group is integrated in the CIBERER (the Spanish Network of Research in Rare Diseases), the COST Myelinet action and the FP7 Leukotreat Project.

Last Updated on Monday, 30 May 2011 08:51

Diabetes Drug Shows Promise in Treatment of Neurodegenerative Disease

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June 6, 2013

Researchers in Spain have found that a drug used to control Type II diabetes can help repair the spinal cords of mice suffering from the inherited disease adrenoleukodystrophy which, untreated, leads eventually to a paralysis, a vegetative state and death. They believe that their findings may be relevant to other neurodegenerative diseases. A Phase II trial will be starting shortly. (see paper).

Last Updated on Monday, 03 February 2014 10:47

We open HMG in August!

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Cover: Proposed molecular mechanism for mitochondrial impairment in X-linked adrenoleukodystrophy. As C26:0 cannot enter peroxisomes for degradation due to the loss of the peroxisomal transporter ABCD1, it accumulates intracellularly affecting the inner mitochondrial membrane impermeability by unknown mechanisms. This alteration may allow a proton flux into the matrix decreasing Δψm, together with a certain extent of electron leakage promoting ROS formation. These ROS oxidise mitochondrial proteins of TCA cycle and OXPHOS, leading to impaired bioenergetics and respiration, and also oxidise mtDNA, contributing to a devil cycle of mitochondrial dysfunction and ultimately, cell demise. See article by López-Erauskin et al., pp. 3296–3305.

Last Updated on Tuesday, 30 July 2013 09:02

La pioglitazona sería útil en adrenoleucodistrofia ligada al cromosoma X

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From: Diario médico, June 10, 2013

El grupo de investigación en enfermedades neurodegenerativas del Instituto de Investigación Biomédica de Bellvitge (Idibell), dirigido por Aurora Pujol, investigadora Icrea, ha demostrado que la pioglitazona, un fármaco para controlar la diabetes tipo 2, es capaz de reparar la médula espinal de modelos de ratones con adrenoleucodistrofia ligada al cromosoma X (ALD-X). (ver el artículo).

Last Updated on Monday, 03 February 2014 10:47

La ciclofilina D se perfila como nueva diana en adrenoleucodistrofia ligada al X

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February 28, 2013 
Javier Granada

Dos trabajos publicados en la revista Brain por un equipo del Idibell, de Barcelona, apuntan a una nueva diana que parece revertir síntomas asociados a la adrenoleucodistrofia ligada al cromosoma X. Derivada del estudio de los fibroblastos, la ciclofilina D parece revertir síntomas ligados a la adrenoleucodistrofia ligada al cromosoma X (X-ALD) (ver el artículo). El equipo de Aurora Pujol ha publicado este mismo lunes un segundo trabajo. En él se describe que el inmunoproteasoma, además de responsable de los procesos inflamatorios, se activa con el estrés oxidativo crónico (ver el artículo).

Last Updated on Tuesday, 30 July 2013 09:04
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