The mechanisms of neurodegeneration in the CAG repeat polyglutamine diseases,
including Spinal and Bulbar Muscular Atrophy (SBMA), Huntington's disease (HD) …

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. In 1993
the mutation that causes HD was identified as an unstable expansion of CAG repeats in the …

Proteolytic processing of mutant huntingtin (mhtt) is regarded as a key event in the
pathogenesis of Huntington's disease (HD). Mhtt fragments containing a polyglutamine …

Huntington's disease (HD) is an autosomal–dominant neurodegenerative disorder caused
by a CAG trinucleotide repeat expansion in the HD gene. The expanded repeats are …

Polyglutamine expansion causes Huntington disease (HD) and at least seven other
neurodegenerative diseases. In HD, N-terminal fragments of huntingtin with an expanded …

The formation of insoluble protein aggregates in neurons is a hallmark of neurodegenerative
diseases caused by proteins with expanded polyglutamine (polyQ) repeats. However, the …

Huntington's disease (HD) is characterized by a progressive loss of neurons in the striatum
and cerebral cortex and is caused by a CAG repeat expansion in the gene encoding …

In Huntington's disease (HD), CAG repeats extend a glutamine tract in huntingtin to initiate
the dominant loss of striatal neurons and chorea. Neuropathological changes include the …

The cellular localization and development of neuronal intranuclear inclusions (NIIs) in cortex
and striatum of R6/2 HD transgenic mice were studied to ascertain the relationship of NIIs to …

One of the most surprising discoveries of the past decade (at least in the field of
neurodegeneration) was that protein misfolding underlies several seemingly disparate …