Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

Neuropathological Classification of Huntingtonʼs Disease

1985 • 2,517 citations

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

1997 • 2,184 citations

Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo

1997 • 1,283 citations

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

1994 • 1,065 citations

Trinucleotide repeat length instability and age of onset in Huntington's disease

1993 • 1,062 citations

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

1995 • 736 citations

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

1995 • 646 citations

Evidence for Degenerative and Regenerative Changes in Neostriatal Spiny Neurons in Huntington's Disease

1985 • 614 citations

A huntingtin-associated protein enriched in brain with implications for pathology

1995 • 597 citations

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

1996 • 580 citations

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH

1996 • 435 citations

Morphometric Demonstration of Atrophic Changes in the Cerebral Cortex, White Matter, and Neostriatum in Huntington's Disease

1988 • 414 citations

Distinct 19 S and 20 S subcomplexes of the 26 S proteasome and their distribution in the nucleus and the cytoplasm.

1994 • 395 citations

Huntingtin Is Ubiquitinated and Interacts with a Specific Ubiquitin-conjugating Enzyme

1996 • 365 citations

Dynamic alterations occur in the levels and composition of transcription factor AP-1 complexes after seizure

1989 • 355 citations

Huntingtin localization in brains of normal and Huntington's disease patients

1997 • 338 citations

Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

1991 • 308 citations

Correlation between the onset age of Huntington's disease and length of the trinucleotide repeat in IT-15

1993 • 231 citations

Glutamine repeats and inherited neurodegenerative diseases: molecular aspects

1996 • 206 citations

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: Relevance to diseases of the nervous system

1996 • 200 citations

Degeneration of pyramidal projection neurons in Huntington's disease cortex

1990 • 193 citations

The complex pathology of trinucleotide repeats

1997 • 191 citations

Expression of Normal and Mutant Huntingtin in the Developing Brain

1996 • 187 citations

CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain

1995 • 180 citations

Nuclear bodies (NBs): A newly “rediscovered” organelle

1992 • 178 citations

Fast transport and retrograde movement of huntingtin and HAP 1 in axons

1997 • 137 citations

Huntington's disease in children

1973 • 97 citations

The cortical neuritic pathology of Huntington's disease

1995 • 89 citations

N-methyl-d-aspartate receptor activation in the neostriatum increases c-fos and fos-related antigens selectively in medium-sized neurons

1991 • 76 citations

Neuronal intranuclear inclusion disease in identical twins

1984 • 72 citations

Inhibition of proteolysis blocks anterograde-retrograde conversion of axonally transported vesicles

1988 • 58 citations

Neuronal intranuclear hyaline inclusion disease: report of a case and review of the literature.

1990 • 49 citations

Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: a pathological correlate of dementia?

1993 • 31 citations