Polyglutamine-Expanded Human Huntingtin Transgenes Induce Degeneration of Drosophila Photoreceptor Neurons

Apoptosis: A Basic Biological Phenomenon with Wideranging Implications in Tissue Kinetics

1972 • 15,349 citations

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

1993 • 7,941 citations

Cell Death: The Significance of Apoptosis

1980 • 7,281 citations

The Genome of Drosophila Melanogaster

1992 • 3,126 citations

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

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

1997 • 2,184 citations

Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1

1993 • 1,596 citations

Transposition of Cloned P Elements into Drosophila Germ Line Chromosomes

1982 • 1,560 citations

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

1997 • 1,283 citations

Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2

1996 • 1,081 citations

Expression of baculovirus P35 prevents cell death in Drosophila

1994 • 1,037 citations

Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

1995 • 843 citations

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

1996 • 824 citations

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

1997 • 821 citations

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

1995 • 774 citations

Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion

1997 • 752 citations

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT

1996 • 745 citations

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

1995 • 736 citations

Inactivation of the Mouse Huntington's Disease Gene Homolog Hdh

1995 • 705 citations

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

1998 • 659 citations

Huntington's Disease

1979 • 607 citations

Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models

1995 • 604 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

Huntingtons Disease

1986 • 551 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

Control of Programmed Cell Death by the Baculovirus Genes p35 and iap

1994 • 517 citations

Widespread expression of Huntington's disease gene (IT15) protein product

1995 • 516 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

Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I

1993 • 473 citations

grim, a novel cell death gene in Drosophila.

1996 • 414 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

Cell Killing by the Drosophila Gene reaper

1996 • 375 citations

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

1997 • 374 citations

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

1998 • 360 citations

Control of programmed cell death by the baculovirus genes p35 and iap.

1994 • 357 citations

In situ evidence for DNA fragmentation in Huntingtonʼs disease striatum and Alzheimerʼs disease temporal lobes

1995 • 350 citations

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

1997 • 338 citations

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

1998 • 338 citations

Huntington's disease : a disorder of families

1989 • 324 citations

The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular Toxicity

1998 • 313 citations

Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies.

1995 • 299 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

Structure and expression of the Huntington's disease gene: Evidence against simple inactivation due to an expanded CAG repeat

1994 • 266 citations

Drosophila Photoreceptor Axon Guidance and Targeting Requires the Dreadlocks SH2/SH3 Adapter Protein

1996 • 248 citations

The embryonic development of the Drosophila visual system

1993 • 214 citations

Baculovirus p35 prevents developmentally programmed cell death and rescues a ced-9 mutant in the nematode Caenorhabditis elegans.

1994 • 204 citations

Characterization and localization of the Huntington disease gene product

1993 • 202 citations

Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)

1994 • 202 citations

Single sperm analysis of the trinucleotide repeats in the Huntington's disease gene: quantification of the mutation frequency spectrum

1995 • 179 citations

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

1998 • 163 citations

Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats

1998 • 161 citations

Blocking apoptosis prevents blindness in Drosophila retinal degeneration mutants

1998 • 157 citations

Induction in the developing compound eye of Drosophila: Multiple mechanisms restrict R7 induction to a single retinal precursor cell

1991 • 155 citations

Pupil and Pseudopupil in the Compound Eye of Drosophila

1972 • 140 citations

Retinal degeneration caused by dominant rhodopsin mutations in Drosophila

1995 • 135 citations

Deletion of Huntington's disease-linked G8 (D4S10) locus in Wolf–Hirschhorn syndrome

1985 • 128 citations

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

1998 • 118 citations

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

1997 • 113 citations

Absence of Disease Phenotype and Intergenerational Stability of the Cag Repeat in Transgenic Mice Expressing the Human Huntington Disease Transcript

1996 • 111 citations

Marked phenotypic heterogeneity associated with expansion of a CAG repeat sequence at the spinocerebellar ataxia 3/Machado-Joseph disease locus.

1995 • 107 citations

Molecular cloning of a full-length cDNA for dentatorubral-pallidoluysian atrophy and regional expressions of the expanded alleles in the CNS.

1995 • 81 citations

Huntington's disease: CAG genetics expands neurobiology

1995 • 64 citations

Evidence from antibody studies that the CAG repeat in the Huntington disease gene is expressed in the protein

1995 • 62 citations

Virus-Like Particles in Normal and Tumorous Tissues of Drosophila

1967 • 54 citations

Mutations in American families with spinocerebellar ataxia (SCA) type 3

1996 • 52 citations

Spinocerebellar ataxia, type 3 (SCA3) is genetically identical to Machado-Joseph disease (MJD)

1995 • 46 citations

The ultrastructure of imaginal disc cells in primary cultures and during cell aggregation in continuous cell lines

1990 • 38 citations

Embryonic development of the antennal lobes of a hemimetabolous insect, the cockroach periplaneta americana: Light and electron microscopic observations

1995 • 36 citations

The development of the Drosophila visual system

1998 • 33 citations

Production of virus-like particles by the transposable genetic element, copia, of Drosophila melanogaster

1987 • 31 citations

The fatal attraction of polyglutamine-containing proteins

1998 • 30 citations

Programmed cell death in Drosophila

1994 • 28 citations

THE COMPOUND EYE

1980 • 22 citations

Nuclear Inclusions: A Study of Aging in Drosophila

1976 • 10 citations

Neuronal cell death

1998 • 4 citations

The compound eye in the opaque-eye phenotype ofDrosophila melanogaster

1975 • 2 citations