Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor

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

1991 • 2,763 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

Glutamine Repeats and Neurodegeneration

2000 • 1,309 citations

Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease

2001 • 1,293 citations

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

1997 • 1,283 citations

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

2001 • 1,218 citations

The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function

1994 • 1,167 citations

Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity

2001 • 1,085 citations

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

1994 • 1,065 citations

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

2000 • 1,033 citations

Cellular Defenses against Unfolded Proteins

2001 • 979 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70

1999 • 830 citations

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

1997 • 821 citations

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

2000 • 722 citations

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: Implications for Huntington’s disease pathology

1999 • 716 citations

Identification of genes that modify ataxin-1-induced neurodegeneration

2000 • 659 citations

Transcriptional Activation Modulated by Homopolymeric Glutamine and Proline Stretches

1994 • 598 citations

Genetic Suppression of Polyglutamine Toxicity in Drosophila

2000 • 562 citations

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

2000 • 553 citations

CREB-binding protein sequestration by expanded polyglutamine

2000 • 540 citations

Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice

1999 • 507 citations

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

2001 • 489 citations

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

1999 • 451 citations

Polyglutamine-Expanded Androgen Receptors Form Aggregates That Sequester Heat Shock Proteins, Proteasome Components and SRC-1, and Are Suppressed by the HDJ-2 Chaperone

1999 • 448 citations

Covalent modification of the androgen receptor by small ubiquitin-like modifier 1 (SUMO-1)

2000 • 448 citations

Evidence for a repressive function of the long polyglutamine tract in the human androgen receptor: possible pathogenetic relevance for the (CAG)_n-expanded neuronopathies

1995 • 446 citations

Phosphorylation of human progesterone receptors at serine-294 by mitogen-activated protein kinase signals their degradation by the 26S proteasome

2000 • 444 citations

Androgen receptor phosphorylation, turnover, nuclear transport, and transcriptional activation. Specificity for steroids and antihormones.

1992 • 430 citations

Analysis of the Role of Heat Shock Protein (Hsp) Molecular Chaperones in Polyglutamine Disease

1999 • 419 citations

Evidence for Proteasome Involvement in Polyglutamine Disease: Localization to Nuclear Inclusions in SCA3/MJD and Suppression of Polyglutamine Aggregation in vitro

1999 • 406 citations

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

2000 • 400 citations

X-LINKED RECESSIVE BULBOSPINAL NEURONOPATHY

1989 • 391 citations

Wild-Type Huntingtin Protects from Apoptosis Upstream of Caspase-3

2000 • 389 citations

p300 and p300/cAMP-response Element-binding Protein-associated Factor Acetylate the Androgen Receptor at Sites Governing Hormone-dependent Transactivation

2000 • 389 citations

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1

2000 • 375 citations

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

1998 • 360 citations

Reduced transcriptional regulatory competence of the androgen receptor in X–linked spinal and bulbar muscular atrophy

1993 • 354 citations

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

2000 • 320 citations

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

1998 • 317 citations

Aberrant Interactions of Transcriptional Repressor Proteins with the Huntington's Disease Gene Product, Huntingtin

1999 • 299 citations

Huntingtin Is Present in the Nucleus, Interacts with the Transcriptional Corepressor C-terminal Binding Protein, and Represses Transcription

2002 • 282 citations

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

2001 • 274 citations

Inhibition of p160-mediated coactivation with increasing androgen receptor polyglutamine length

2000 • 271 citations

Histone deacetylase inhibitors reduce polyglutamine toxicity

2001 • 244 citations

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

2002 • 235 citations

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

1995 • 223 citations

The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: Neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis

2001 • 187 citations

Ubc9 Interacts with the Androgen Receptor and Activates Receptor-dependent Transcription

1999 • 181 citations

A family with adult spinal and bulbar muscular atrophy, X-linked inheritance and associated testicular failure

1983 • 170 citations

Huntingtin's Neuroprotective Activity Occurs via Inhibition of Procaspase-9 Processing

2001 • 164 citations

The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract

2001 • 155 citations

Altered transcription in yeast expressing expanded polyglutamine

2001 • 125 citations

Changes in the Abundance of Androgen Receptor Isotypes: Effects of Ligand Treatment, Glutamine-Stretch Variation, and Mutation of Putative Phosphorylation Sites

1994 • 105 citations

Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy

2001 • 97 citations

Transglutaminase-catalyzed inactivation of glyceraldehyde 3-phosphate dehydrogenase and α-ketoglutarate dehydrogenase complex by polyglutamine domains of pathological length

1997 • 96 citations

Evidence for a recruitment and sequestration mechanism in Huntington'sdisease

1999 • 95 citations

A Cell Culture Model for Androgen Effects in Motor Neurons

1998 • 94 citations

Embryonic mouse spinal cord motor neuron hybrid cells

1991 • 94 citations

Polyglutamine Domains Are Substrates of Tissue Transglutaminase: Does Transglutaminase Play a Role in Expanded CAG/Poly‐Q Neurodegenerative Diseases?

1997 • 92 citations

Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome)

1988 • 92 citations

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

1997 • 89 citations

X‐linked spinomuscular atrophy

1992 • 83 citations

Molecular cloning of rat prostate transglutaminase complementary DNA. The major androgen-regulated protein DP1 of rat dorsal prostate and coagulating gland.

1992 • 79 citations

ADAM7, A Member of the ADAM (A Disintegrin And Metalloprotease) Gene Family Is Specifically Expressed in the Mouse Anterior Pituitary and Epididymis¹

1997 • 71 citations

Tissue-specific and androgen-regulated expression of human prostate-specific transglutaminase

1996 • 71 citations

Expression and Regulation of Metallothioneins in the Rat Epididymis

2001 • 50 citations

Androgen receptor mutation in Kennedy'sdisease

1999 • 47 citations

Effects of Sex Steroids on Secretory Granule Formation in Gonadotropes of Castrated Male Rats with Respect to Granin Expression*

1998 • 37 citations

Androgens Regulate the Mammalian Homologues of Invertebrate Sex Determination Genes tra-2 and fox-1

2001 • 28 citations

Developmental and hormonal regulation of type II DNA topoisomerase in rat testis

2001 • 25 citations

Characterization of an Androgen-Specific Response Region Within the 5′ Flanking Region of the Murine Epididymal Retinoic Acid Binding Protein Gene1

2000 • 23 citations

Regulation of Na+,K+-ATPase in submandibular glands of hypophysectomized male mice by steroid and thyroid hormones.

1996 • 21 citations

Glucocorticoid, androgen, and retinoic acid regulation of glutathione S-transferase gene expression in hamster smooth muscle tumor cells

1992 • 5 citations