Metabolic Rewiring Modulates Mutant Huntingtin Aggregation in Huntingtons Disease

Abstract

Huntington s disease (HD) is an inherited and progressive neurodegenerative newlinedisorder characterized by motor, cognitive and behavioural/psychiatric newlineabnormalities. HD is caused by the expansion of CAG repeats (gt39) on the newlineHuntingtin gene coding for mutant Huntingtin protein with a long polyglutamine newlinetract which aggregates leading to neuronal death. In addition, multiple factors newlinesuch as age, genetic modifiers, gene polymorphism and cellular mechanisms newlinelike protein aggregation, mitochondrial dysfunction and oxidative stress, could newlinemodify the course of the disease progression, however, their precise newlinemechanism is not well understood. newline