Glutamine repeats and neurodegenerative diseases: molecular aspects

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

1993 • 7,941 citations

α-Synuclein in filamentous inclusions of Lewy bodies from Parkinson’s disease and dementia with Lewy bodies

1998 • 2,898 citations

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

1991 • 2,763 citations

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

1997 • 2,762 citations

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

1997 • 2,184 citations

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

1998 • 1,592 citations

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the α1A-voltage-dependent calcium channel

1997 • 1,571 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

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

1997 • 821 citations

Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy

1998 • 764 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

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

1995 • 646 citations

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

1996 • 580 citations

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

1997 • 528 citations

Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

1997 • 513 citations

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

1998 • 490 citations

Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse

1997 • 374 citations

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

1996 • 365 citations

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

1998 • 338 citations

Inhibiting transthyretin amyloid fibril formation via protein stabilization

1996 • 338 citations

Episodic Ataxia Type 2 (EA2) and Spinocerebellar Ataxia Type 6 (SCA6) Due to CAG Repeat Expansion in the CACNA1A Gene on Chromosome 19p

1997 • 288 citations

Nuclear inclusion in oculopharyngeal dystrophy

1980 • 244 citations

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

1995 • 223 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

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

1998 • 191 citations

SCA6 is caused by moderate CAG expansion in the alpha1A-voltage- dependent calcium channel gene

1997 • 113 citations

Transgenic models of Huntington's disease

1997 • 112 citations

Neural Precursor Cells: Applications for the Study and Repair of the Central Nervous System

1997 • 106 citations

Review

1996 • 96 citations

Characterization of an Expanded Glutamine Repeat Androgen Receptor in a Neuronal Cell Culture System

1997 • 89 citations

Partial characterisation of murine huntingtin and apparent variations in the subcellular localisation of huntingtin in human, mouse and rat brain

1996 • 85 citations

Identification of a self-association region within the SCA1 gene product, ataxin-1

1997 • 63 citations