Recruitment and the Role of Nuclear Localization in Polyglutamine-mediated Aggregation

Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice

1996 • 3,012 citations

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

1997 • 2,762 citations

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

1994 • 1,698 citations

Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear Inclusions

1998 • 1,592 citations

Hsp104, Hsp70, and Hsp40

1998 • 1,346 citations

Coiled coils: new structures and new functions

1996 • 1,196 citations

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

1994 • 1,065 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Structure and Function in the Nucleus

1998 • 925 citations

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

1997 • 821 citations

Coiled coils: new structures and new functions

1996 • 812 citations

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1

1998 • 730 citations

Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in Drosophila

1998 • 659 citations

Transcriptional Activation Modulated by Homopolymeric Glutamine and Proline Stretches

1994 • 598 citations

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

1995 • 582 citations

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

1996 • 580 citations

Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract

1998 • 569 citations

The eyes absent gene: Genetic control of cell survival and differentiation in the developing Drosophila eye

1993 • 557 citations

Expanded polyglutamine in the Machado–Joseph disease protein induces cell death in vitro and in vivo

1996 • 546 citations

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

1997 • 528 citations

Spinocerebellar ataxia 3 and machado‐joseph disease: Clinical, molecular, and neuropathological features

1996 • 430 citations

Intranuclear Neuronal Inclusions: A Common Pathogenic Mechanism for Glutamine-Repeat Neurodegenerative Diseases?

1997 • 392 citations

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture

1998 • 376 citations

Identification and characterization of the gene causing type 1 spinocerebellar ataxia

1994 • 369 citations

Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch

1998 • 360 citations

Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions

1998 • 338 citations

Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

1998 • 328 citations

Trinucleotide Repeats in Neurogenetic Disorders

1996 • 323 citations

Genetic instabilities and hereditary neurological diseases

1998 • 268 citations

Machado‐Joseph disease gene product is a cytoplasmic protein widely expressed in brain

1997 • 265 citations

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases.

1995 • 223 citations

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

1996 • 200 citations

Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy

1998 • 191 citations

Hsp70 and Hsp40 Chaperone Activities in the Cytoplasm and the Nucleus of Mammalian Cells

1997 • 181 citations

Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

1998 • 163 citations

A 70 Amino Acid Region within the Semaphorin Domain Activates Specific Cellular Response of Semaphorin Family Members

1997 • 145 citations

Wag the Tail: Structural Dynamics of Actomyosin

1998 • 140 citations

A clinical and pathologic study of a large Japanese family with Machado‐ Joseph disease tightly linked to the DNA markers on chromosome 14q

1994 • 134 citations

Trinucleotide expansion within the MJD1 gene presents clinically as spinocerebellar ataxia and occurs most frequently in German SCA patients

1995 • 134 citations

Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia.

1995 • 133 citations

Ataxin-3 is transported into the nucleus and associates with the nuclear matrix

1998 • 118 citations

Multiple Roles of theeyes absentGene inDrosophila

1998 • 111 citations

Molecular and clinical correlations in spinocerebellar ataxia type 3 and Machado‐Joseph disease

1995 • 99 citations

Joseph's disease: Clinical and pathological studies in a Japanese family

1986 • 88 citations

Dentatorubral Pallidoluysian Atrophy (DRPLA) Protein Is Cleaved by Caspase-3 during Apoptosis

1997 • 79 citations

Oligomerization of Expanded-Polyglutamine Domain Fluorescent Fusion Proteins in Cultured Mammalian Cells

1997 • 66 citations

Joseph disease: A multisystem degenerative disorder of the nervous system

1982 • 52 citations

Polyglutamines, nuclear inclusions and neurodegeneration

1997 • 52 citations

Machado–Joseph Disease Gene Product Identified in Lymphocytes and Brain

1997 • 43 citations

The fatal attraction of polyglutamine-containing proteins

1998 • 30 citations