Mitochondrial Dynamics and Huntington’s Disease: A Dance of Fate

Abstract

Huntington's disease (HD) is a devastating neurodegenerative disorder whose age of onset can vary quite widely, with the most severe forms striking as early as the first decade of life. The disease is caused by an abnormal expansion of a polyglutamine tract in the huntingtin (hit) protein. The mechanism by which polyglutamine expansions in htt cause disease is still not understood, although many different mechanisms have been proposed. One theory is that HD is caused by an impairment of mitochondria function. Mitochondria are vital for cell survival. They are the main powerhouse for ATP generation and are the repository of many important molecules that govern whether a cell lives or dies. Studies have shown that mitochondria are not static structures, but are highly dynamic, undergoing constant cycles of fusion and fission. Accumulating evidence suggests that HD is associated with a disturbance of mitochondrial dynamics. Here we review the published data linking mitochondrial dysfunction in HD, concentrating on the role that defects in mitochondrial dynamics might play in the disease.