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

17th national competition for scientific and technical research

Rare diseases

Senior Researcher : José María González-Granado

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


The 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.

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