Huntington's disease (HD) is a fatal neurodegenerative condition caused by expansion of
the polyglutamine tract in the huntingtin (Htt) protein. Neuronal toxicity in HD is thought to be …

The staggerer mutation was first identified at the Jackson Laboratory in 1955. In the ensuing
half-century, studies of staggerer mice have provided new insights into developmental …

Huntington's disease (HD) is a devastating neurodegenerative disorder that occurs in
patients with a mutation in the huntingtin or IT15 gene. Patients are plagued by early …

Heat shock proteins (hsps) are protective against the harmful effects of mutant expanded
polyglutamine repeat proteins that occur in diseases such as Huntington's, prompting the …

Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disorder caused by abnormal
expansions of a trinucleotide CAG repeat in exon 47 of the CACNA1A gene, which encodes …

A growing number of human neurodegenerative diseases are associated with the expres-
ion of misfolded proteins that oligomerize and form aggregate structures. Over time …

Background Huntington's disease, spinal and bulbar muscular atrophy, and spinocerebellar
ataxia 17 (SCA17) are caused by expansions in the polyglutamine (polyQ) repeats in …

Abstract Background DAX1 (NR0B1), a member of the nuclear receptors super family, has
been shown to be involved in the genetic sex determination and in gonadal differentiation in …

Elucidating the mechanisms of alternative splicing in the brain is a prerequisite to the
understanding of the pathogenesis of major neurological diseases linked to impairment of …

Spinocerebellar ataxia-1 (SCA1) is caused by the expansion of a polyglutamine repeats
within the disease protein, ataxin-1. The mutant ataxin-1 precipitates as large intranuclear …