It has been five years since the elucidation of the genetic mutation underlying the
pathogenesis of Huntington's disease (HD)(97), however the precise mechanism of the …

Neurodegenerative disorders, which are chronic and progressive, are characterized by
selective and symmetric loss of neurons in motor, sensory, or cognitive systems. Delineation …

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 …

Huntington's disease is a progressive neurodegenerative disorder caused by a
polyglutamine [poly (Q)] repeat expansion in the first exon of the huntingtin protein …

Huntington's disease (HD) is an inherited, neurodegenerative disorder caused by the
expansion of a glutamine repeat in the N-terminus of the huntingtin protein. To gain insight …

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 mouse autosomal dominant mutation Mody develops hyperglycemia with notable
pancreatic β-cell dysfunction. This study demonstrates that one of the alleles of the gene for …

At least eight inherited human neurodegenerative diseases are caused by expansion of a
polyglutamine domain within the respective proteins 1, 2. This confers dominant toxicity on …

Polyglutamine (polygln) diseases are a group of inherited neurodegenerative disorders
characterized by protein misfolding and aggregation. Here, we investigate the role in polygln …

Spinocerebellar ataxia type 3, also known as Machado-Joseph disease (SCA3/MJD), is one
of at least eight inherited neurodegenerative diseases caused by expansion of a …