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Projects. Life and Earth Sciences

Chronic activation of the TGF‑beta route in Marfan's syndrome: deregulation of intracellular traffic and the extracellular matrix

Lead Researcher:
Gustavo Egea Guri

Research Centre:
Instituto de Investigaciones Biomédicas August Pi i Sunyer (IDIBAPS). Barcelona.

Synopsis: 

Gustavo Egea GuriThe aim is to use cellular models of Marfan's syndrome to investigate the contribution of different routes for the internalisation of the TGF β complex and its receptor, together with the potential differential expression of integrins as these complex essential proteins mediate in the communication between cells and the extracellular matrix synthesising and surrounding them; this is primarily damaged by mutation in fibrilin-1, which forms part of the elastic fibres. Alterations in both processes would contribute significantly to the characteristic chronic TGF β signalling typically occurring in this disease. It has been found that the molecular mechanism associated with the internalisation route through lipid rafts/caveoline presents a reduction in the levels of expression of some of its essential components. This means that the signalling which leads under normal conditions to the extinction of the signalling by TGF β is altered in the vascular smooth muscle cells of Marfan patients, so that signalling by TGF β will not have the necessary and expected effect. As a result, this cytokine eventually causes permanent activation in these cells. On the other hand, Marfan cells were found to have altered capacities for adhesion and migration, directly associated with alterations in the organisation of the actin cytoskeleton and activation of members of the Rho GTPase family.

Scientific production
2 papers at international conferences

Researcher's web address:

 http://www.ub.edu/sgr120/welcome.htm



Gustavo Egea Guri

Professor of Cellular Biology in the Medical Faculty of the University of Barcelona, and a research member of the August Pi i Sunyer Biomedical Research Institute (IDIBAPS) and the Institute of Nanosciences and Nanotechnology (IN2UB) of the University of Barcelona. Spokesman and treasurer of the Spanish Cellular Biology Society (SEBC). In his scientific career he has studied the mechanism of acetylcholine liberation, the subcellular compartmentation of protein glycosylation processes in tumour cells, and the molecular mechanisms determining the architecture of Golgi's apparatus and how it interacts with the actin cytoskeleton in eukaryote cells. Recently he commenced a new line of research into the pathophysiology of Marfan's syndrome, centring on the traffic of receptors for TGF‑b and the assembly of the extracellular matrix in vascular smooth muscle cells from the aorta of murine models and patients affected by Marfan's syndrome. He was awarded the Prize for Young University Researcher (2002) and the August Pi i Sunyer Prize (2010) by the Catalonia Regional Government. He has worked in several national and international centres, such as the Biozentrum of the University of Basel (1987-88), the Severo Ochoa Molecular Biology Centre (Madrid, 1993), the European Molecular Biology Laboratory (Heidelberg, 2001), the Mario Negri Sud Institute (Chieti, 1998) and the Curie Institute (Paris, 2009). His teaching activity centres on Histology and Microscopic Organography in the degrees of Medicine and Biomedical Engineering, and in several master's degrees in the University of Barcelona.


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