Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington
disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal …

Two decades ago, molecular genetic analysis provided a new approach for defining the
roots of inherited disorders. This strategy has proved particularly powerful because, with …

Huntington's disease is characterized by a loss of brain striatal neurons that occurs as a
consequence of an expansion of a CAG repeat in the huntingtin protein. The resulting …

Huntington's disease (HD) is characterized by the selective loss of striatal projection
neurons. In early stages of HD, neurodegeneration preferentially occurs in the lateral globus …

Huntington's disease (HD) is a late manifesting neurodegenerative disorder in humans
caused by an expansion of a CAG trinucleotide repeat of more than 39 units in a gene of …

To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage
have important effects on disease-related transcriptional dysfunction, we compared the …

Huntington's disease (HD) is a neurodegenerative disorder caused by a polyglutamine
(polyQ) tract expansion near the N terminus of huntingtin (Htt). Proteolytic processing of …

Although the gene responsible for Huntington's disease was discovered in 1993, the
pathogenic mechanisms by which mutant huntingtin causes neuronal dysfunction and death …

The corticostriatal pathway provides most of the excitatory glutamatergic input into the
striatum and it plays an important role in the development of the phenotype of Huntington's …

Huntington's disease (HD) is a late-onset neurodegenerative disease for which the mutation
is CAG/polyglutamine repeat expansion. The R6 mouse lines expressing the HD mutation …