Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder with midlife
onset characterized by psychiatric, cognitive, and motor symptoms. Death occurs between …

Huntington's disease (HD) is one of an increasing number of human neurodegenerative
disorders caused by a CAG/polyglutamine-repeat expansion. The mutation occurs in a gene …

The discovery that expansion of unstable repeats can cause a variety of neurological
disorders has changed the landscape of disease-oriented research for several forms of …

The mechanism by which an elongated polyglutamine sequence causes neurodegeneration
in Huntington's disease (HD) is unknown. In this study, we show that the proteolytic cleavage …

The mechanisms by which mutant huntingtin induces neurodegeneration were investigated
using a cellular model that recapitulates features of neurodegeneration seen in Huntington's …

Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the
gene encoding huntingtin protein (Htt), but the mechanisms by which this mutant protein …

The data we report in this study concern the types, location, numbers, forms, and
composition of microscopic huntingtin aggregates in brain tissues from humans with different …

We have produced yeast artificial chromosome (YAC) transgenic mice expressing normal
(YAC18) and mutant (YAC46 and YAC72) huntingtin (htt) in a developmental and tissue …

The mechanism of neurodegeneration in CAG/polyglutamine repeat expansion diseases is
unknown but is thought to occur at the protein level. Here, in studies of spinocerebellar …