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Projects. Life and Matter Sciences

Visualisation of neuronal polysomal architecture and its alterations in Huntington's Disease

Lead Researcher: José Jesús Fernández Rodríguez
Research Centre: National Center for Biotechnology, Spanish National Research Council (CNB-CSIC).


José Jesús Fernández RodríguezHuntington's disease (HD) is an inherited disorder that involves the selective degeneration of the striatal medium-sized spiny neurons and, at later stages, of cerebral cortex neurons. The disease is clinically characterized by motor, cognitive and psychiatric manifestations. Onset occurs, on average, around the age of 40 years and it is fatal after 15-20 years of inexorable, progressive deterioration. Despite the extensive research since the discovery of its genetic cause, the precise pathophysiological mechanisms of HD are poorly understood and there is still no effective treatment for the disease. Our working hypothesis is that the maintenance of the subcellular architecture is of paramount importance for neuronal homeostasis. Identification of alterations associated to neurodegeneration may thus contribute to gain insights into the pathophysiological basis of different diseases and enable the identification of new targets for therapeutic intervention. In this project we are studying the architecture of polysomes, which are responsible for satisfying the high demands of protein synthesis in neurons. Our aim is the identification and characterization of polysomal alterations in HD and the exploration of the therapeutic potential of molecules that may reverse them. As central tools, we use electron tomography -whose principles are similar to computed tomography commonly used in Medicine- and advanced image processing methods, in combination with protocols for optimal structural preservation of brain tissue that we have developed. This strategy enables three-dimensional visualization of the in situ neuronal subcellular architecture at close-to-molecular resolution and, thus, the analysis of the neuronal protein synthesis machinery.

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