ABSTRACT
Huntington's disease is an autosomal dominantly inherited debilitating disorder caused by a cytosine-adenine-guanine triplet codon repeat of variable length. Although the length of cytosine-adenine-guanine (CAG) repeats can be used for predicting the age of onset, the CAG repeat length seems to contribute less to the rate of progression; understanding the determinants of the rate of progression could help find targets for therapeutic intervention. Mitochondria are dynamic organelles that undergo controlled fusion and fission processes, and they move along the cytoskeleton in neurons in an anterograde and retrograde manner. Mitochondria replicate using binary fission in a process known as mitochondrial biogenesis which is under stringent control by several nuclear-encoded transcription factors, coactivators, and repressors. Regular injections of ascorbate, the deprotonated form of vitamin C, beginning at symptom onset restored the behavior-related release of ascorbate in the striatum and improved behavioral responses in R6/2 mice.
