CONTEXT

Huntington's disease (HD) is a neurodegenerative genetic disorder that usually becomes noticeable in middle age. It affects muscle coordination and provokes cognitive decline and dementia. HD is caused by an autosomal dominant mutation of a gene called Huntingtin (Htt) leading to an abnormal repeat number of the amino acid Glutamine (Q) in the protein (PolyQ-Htt). This mutant form of the protein has altered activities and is prone to form protein aggregates. It is toxic to certain types of cells, especially in the brain where neurons are particularly affected.

There is at the present time no cure for HD, and the available treatments aim at reducing the severity of some of its symptoms.