ABSTRACT
Huntington’s disease (HD) is a hereditary neurodegenerative disorder marked by characteristic chorea, dystonia, Parkinsonism, cognitive deterioration, and behavioral changes. Prolongation of the CAG trinucleotide within the IT15 gene has been associated with the development of HD. Cognitive dysfunction develops before loss of motor function with accompanying intense psychological discomfort that leads to psychiatric and behavioral symptoms. With no known treatment currently, patients with HD are offered education and symptomatic management of their condition. The significant role of the loss of HTT, as well as altered autophagy and mitophagy, It has been established in the development of HD. These findings are augmented by the possible involvement of neuroinflammation and oxidative stress in all stages of the development of HD. With advances in modern trends of therapy, such as the use of antisense oligonucleotides and RNA interference compounds that target mRNA, and zinc-finger transcriptional repressors and clustered interspaced short palindromic repeats (CRISPR/Cas9) methods, there is optimism about future prospects in the effective management of this neurodegenerative disorder. This chapter looks with key interest at the prevailing and emerging knowledge on HD with an emphasis on disease progression, varying signaling processes, and current and incipient management options.
