A stable G-quartet binds to a huntingtin protein fragment containing expanded polyglutamine tracks.

Huntington's disease (HD) is a progressive neurodegenerative disorder that is inherited in an autosomal dominant fashion. The disease is the result of an expanded CAG repeat in exon 1 of the HD gene, which encodes an elongated polyglutamine tract in the mutant form of the protein, huntingtin. Disease pathogenesis is linked to intracellular aggregates that form because of the tendency of the mutant protein to misfold.

DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates.

Huntington's disease (HD) is a neurodegenerative disorder that follows an autosomal-dominant inheritance pattern. The pathogenesis of the disease depends on the degree of expansion of triplet (CAG) repeats located in the first exon on the gene. An expanded polyglutamine tract within the protein huntingtin (Htt) enables a gain-of-function phenotype that is often exhibited by a dysfunctional oligomerization process and the formation of protein aggregates.