Huntington’s disease (HD) is an inherited disease that involves selective neurodegeneration of medium-sized spiny neurons of the striatum and subsequently of neurons of the cerebral cortex. One of the major limitations that exist in the study of HD progression is that studies of brain deterioration are limited to the use of non-invasive imaging techniques and the available samples are obviously from post-mortem subjects.

In this project it is proposed to study the subcellular architecture in peripheral tissues in HD with three purposes:

• to compare whether the molecular and subcellular alterations that occur in the brain also occur in peripheral tissues
• use peripheral tissue biopsies to follow biomarkers of the disease based on molecular / cellular alterations
• study whether there are alterations that are specific to peripheral tissues