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Projects. Life and Matter Sciences

New mechanisms for regulating the immune response for lamin A/C and progerine: implications in Hutchinson-Gilford premature ageing syndrome

Lead Researcher:
José María González-Granado

Research Centre:
Centro Nacional de Investigaciones Cardiovasculares (CNIC). Madrid.


José María González-GranadoThe Hutchinson Gilford Syndrome (HGPS) is a rare disease (code OMIM 176670) which affects 1 in every 4-8 million children. It is characterised by accelerated ageing and death at approximately 13 years of age, and there is no cure or effective treatment. This disease is caused by accumulation of mutant lamin A/C, progerine, which also accumulates physiologically in healthy individuals during ageing. Knowledge of the molecular and cellular changes that cause progerine is not only essential in order to establish the origins of the disease, but also to explain some of the causes of natural ageing. We recently published that lamin A/C is synthesised in T lymphocytes following recognition of an antigen to modulate their activation. Nevertheless, the effect of progerine on the activation of T cells is unknown, as is the role of lamin A/C and progerine in other inflammatory responses. Two of the aims of this project involve ascertaining these effects. Another aim is to discover what effect the treatments used have, or the effects of treatments which are thought to be of interest for the treatment of HGPS on the immune system of patients. These objectives have the purpose of improving the lives of HGPS patients and understanding the role of lamin A/C and progerine in immune diseases where these proteins may play an important role.

José María González-Granado

Degree in Biology, Universidad Autónoma de Madrid (UAM) in 1999, he studied for his doctorate (2000-2004) in the Facultad de Medicina of the UAM, investigating the relationship between the function and structure of the arteries and increasing arterial pressure. These studies led to the award of the Premio Extraordinario de Doctorado (2005) and European Doctor mention. Thanks to a Marie Curie European grant, he continued his studies into the vascular wall in the University of Glasgow (UK, 2003-2005). He then went on to join the Instituto de Biomedicina, Valencia (IBV-CSIC, 2005-2009) and was awarded a "Sara Borrell" contract to study the origin of atherosclerosis and the consequences of cellular accumulation of progerine (the mutant lamin A/C protein that causes Hutchinson-Gilford premature ageing syndrome (HGPS)). Since 2009 he has worked in the Centro Nacional de Investigaciones Cardiovasculares (CNIC) with the chief aim of discovering the role of lamin A/C and progerine in the immune response mediated by T cells. Since 2012 he is the "Miguel Servet" Postdoctoral researcher and leads his own research group with the aim of making high quality and translational scientific contributions.

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