Proteasomal dysfunction in aging and Huntington disease

Dysfunction of wild-type huntingtin in Huntington disease.

Huntingtin is the protein involved in Huntington disease (HD), an inherited neurodegenerative disease. Research activities have focused on the abnormal functions of mutant huntingtin. However, recent results indicate that wild-type huntingtin has important activities in brain neurons, suggesting that loss of these activities may play a role in HD.

Elena Cattaneo disease Dysfunction of Wild - Type Huntingtin in Huntington

Int. Union Physiol. Sci./Am.Physiol. Soc.. ESSN: 1548-9221. Visit our website at http://www.the-aps.org/. December by the American Physiological Society, 9650 Rockville Pike, Bethesda MD 20814-3991. © 2003 physiological developments. It is published bimonthly in February, April, June, August, October, and (formerly published as News in Physiological Science) publishes brief review articles on m...

Neuronal Dysfunction in Aging and Alzheimer’s Disease

Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in Huntington disease

Phonatory Dysfunction as a Preclinical Symptom of Huntington Disease

PURPOSE Although dysphonia has been shown to be a common sign of Huntington disease (HD), the extent of phonatory dysfunction in gene positive premanifest HD individuals remains unknown. The aim of the current study was to explore the possible occurrence of phonatory abnormalities in prodromal HD. METHOD Sustained vowel phonations were acquired from 28 premanifest HD individuals and 28 health...