ABSTRACT
Huntington’s disease (HD) is a progressive, fatal, dominant, hereditary, and incurable neurodegenerative disease of the brain in which primarily the striatum and cortex that are involved in movement, intellect, and emotions are gradually destroyed. This disease is characterized by an expansion of more than 35 repeats of the trinucleotide CAG (cytosine-adenine-guanosine) coding for glutamine in huntingtin gene. Among various cellular defects, increased oxidative stress, chronic inflammation, and glutamate levels play an important role in the pathogenesis of HD. This chapter briefly presents incidence and cost, signs and symptoms, brain pathology, receptor abnormalities, microRNAs role pathogenesis, regulation of activation of Nrf2, and Nrf2 in HD. It presents evidence to show that increased oxidative stress, chronic inflammation, and glutamate levels contribute to the initiation and progression of neurodegeneration. It also proposes that in order to simultaneously decrease the above biochemical defects, a concurrent elevation of the levels of antioxidant enzymes together with dietary and endogenous antioxidants and B-vitamins may be necessary. This chapter also suggests a mixture of micronutrients that may simultaneously attenuate these biochemical abnormalities involved in HD, and thereby, prevent, and in combination with standard therapy, improve the management of this disease.
