Autophagy Pathways in Huntington’s Disease

Huntington’s disease (HD) is a hereditary neurodegenerative disorder characterized by motor, cognitive and behavioural abnormalities. HD is caused by a mutation in the huntingtin gene which produces an enlarged chain of CAG triplets in this gene and an expanded chain of poliglutamines in the N terminal portion of the protein. HD is characterized by neuronal loss and atrophy of several brain nuclei, preferentially in the striatum.