Beclin‐1 is a protein that plays a role in autophagy, and in the context of HD, a mutation in the gene encoding ataxin‐3 (ATXN3) has been implicated in the pathogenesis of the disease. Beclin‐1 may be involved in the clearance of mutant ataxin‐3, and ataxin‐3 may play a role in the regulation of ubiquitination and autophagy. This evidence concerns the gene ATXN3 and Huntington disease.