Metabolic dysregulation in Huntington's disease – molecular insights from model organisms

Huntington's disease (HD) is the most common autosomal dominant inherited neurodegenerative disease. Clinically HD is traditionally characterized by a combination of movement disorder, psychiatric abnormalities and cognitive decline. In addition systemic metabolic abnormalities culminating in severe emaciation are increasingly recognized as independent hallmarks of the disease. The monogenetic nature of HD and the availability of a wide range of transgenic model organisms have allowed researchers to dissect the molecular pathology of HD in unprecedented detail. Recent advances suggest that neurodegeneration and…