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Identification of the function of the Wt1gene in Huntington's disease

17th national competition for scientific and technical research

Rare diseases

Senior Researcher : Ofelia M. Martínez Estrada

Research Centre or Institution : Departamento de Biología Celular. Universidad de Barcelona


Huntington's disease (HD) is a neurodegenerative disease caused by the expansion of CAG triplets which code the synthesis of glutamine in the Huntington protein. Several molecular mechanisms have been identified by means of which mutated Huntington (mHtt) is able to produce neuronal toxicity in advanced stages of the disease. Nevertheless, there is practically no information about the molecular mechanisms involved in the toxicity of this in early stages. The Wt1gene codes for a transcription factor that is expressed dynamically during the development of the brain. Recently an increase in its expression has been described in a mouse HD model and in patients with this disease. Our preliminary data suggest that the over-expression of Wt1 in neurons may be associated with an increase in susceptibility to death. During the past 10 years new functions of WT1 have been identified in different tissues and organs, although its role in the brain has yet to be studied. The main aim of our project is to identify the functions of WT1 during brain development and to understand the meaning of its over expression in HD. By understanding the functions of WT1 in brain development we will be able to elucidate its role in the development of HD. The answer to this question is critical and may indicate whether peptide vaccine against WT1 which is currently used in the treatment of cancer may have therapeutic potential in the treatment of HD.

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