Adult-onset neurodegenerative disorders are disabling and often fatal diseases of the
nervous system whose underlying mechanisms of cell death remain unknown. Defects in …

In recent years, several important advances have been made in our understanding of the
pathways that lead to cell dysfunction and death in Parkinson's disease (PD) and …

Polyglutamine expansion (polyQ) in the protein huntingtin is pathogenic and responsible for
the neuronal toxicity associated with Huntington's disease (HD). Although wild-type …

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 …

An expanded CAG repeat is the underlying genetic defect in Huntington disease, a disorder
characterized by motor, psychiatric and cognitive deficits and striatal atrophy associated with …

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 …

A protein required to localize mitochondria to Drosophila nerve terminals has been identified
genetically. Photoreceptors mutant for milton show aberrant synaptic transmission despite …

Huntington's disease (HD) is initiated by an abnormally expanded polyglutamine stretch in
the huntingtin protein, conferring a novel property on the protein that leads to the loss of …

At least nine inherited neurodegenerative diseases share a polyglutamine expansion in
their respective disease proteins. These diseases show distinct neuropathological changes …

Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects
of the respiratory chain, which has helped researchers explain their genetic and clinical …