A Bird’s Eye View on Cellular Dynamics In Huntington’s Disease

Abstract

Huntington's chorea is a rare neurodegenerative disorder caused by the dominant inheritance of the mutated huntingtin gene, housing poly-CAG or polyglutamine repeats. It is characterized by a wide variety of symptoms ranging from chorea and hypokinetic movements to behavioural and cognitive decline, followed by dementia and inevitable death. Over the past 120 years, all available therapeutics have been for the symptomatic management of Huntington's disease (HD) and require supportive physiotherapy and counselling to maximize the efficacy of the treatment. Several animal models have been employed to help elucidate and decrypt the pathophysiology of the disease, and also screen potential therapeutic candidates. In the last few decades, a deeper understanding of the cellular and molecular dynamics associated with HD has helped shed light on the mechanisms involved in disease progression. Genetic intervention for early detection, spreading awareness about HD and its symptoms, and training professionals in the nuances of the disease condition can significantly improve the lifestyle of patients. This article aims at summarizing the complex pathogenesis of HD at the cellular level using various disease models and available therapeutics