Treatments to delay

Huntington disease (HD) is the prototypic disease caused by expansion of unstable GAG repeat. It primarily affects striatal neurons. It is a mid-life onset disorder characterized by unvoluntary movements (chorea), personality changes and dementia that progress to death within 10-20 years of onset. There are currently no treatment to delay or prevent appearance of the symptoms in the patients. Other diseases in this class include spinocerebellar ataxias (SCA) 1, 2, 3 (also known as Machado-Joseph disease, MJD), 6, and 7, DRPLA, and spinobulbar muscular atrophy (SMA, also known as Kennedy s disease) (Zoghbi and Orr 2000). 

A new episode of controlled ripening started with the investigation of ethylene binding site inhibitors, like 1-methylcyclopropene (1-MCP) and norbornadiene (2,5-NBD) (Fig. 11.4), and their development for market use [29]. Meanwhile 1-MCP was commercialized for postharvest treatment to delay ripening of apples [30-32]. 

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