Nucleocytoplasmic trafficking and transcription effects of huntingtin in Huntington's disease

[1]  Michael P. Cusack,et al.  Huntingtin Phosphorylation Sites Mapped by Mass Spectrometry , 2006, Journal of Biological Chemistry.

[2]  K. Shinoda,et al.  Huntingtin-associated protein 1 (HAP1) interacts with androgen receptor (AR) and suppresses SBMA-mutant-AR-induced apoptosis. , 2006, Human molecular genetics.

[3]  U. Stelzl,et al.  Huntingtin interacts with the receptor sorting family protein GASP2 , 2006, Journal of Neural Transmission.

[4]  T. Ebner,et al.  Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8 , 2006, Nature Genetics.

[5]  S. Hersch,et al.  Sp1 Is Up-regulated in Cellular and Transgenic Models of Huntington Disease, and Its Reduction Is Neuroprotective* , 2006, Journal of Biological Chemistry.

[6]  T. Cooper,et al.  RNA-mediated neuromuscular disorders. , 2006, Annual review of neuroscience.

[7]  L. Raymond,et al.  Cleavage at the Caspase-6 Site Is Required for Neuronal Dysfunction and Degeneration Due to Mutant Huntingtin , 2006, Cell.

[8]  K. Jones,et al.  Wnt signaling: is the party in the nucleus? , 2006, Genes & development.

[9]  Lu Gan,et al.  Palmitoylation of huntingtin by HIP14is essential for its trafficking and function , 2006, Nature Neuroscience.

[10]  M. MacDonald,et al.  Decreased association of the transcription factor Sp1 with genes downregulated in Huntington's disease , 2006, Neurobiology of Disease.

[11]  In-Hyun Park,et al.  A Nuclear Transport Signal in Mammalian Target of Rapamycin Is Critical for Its Cytoplasmic Signaling to S6 Kinase 1* , 2006, Journal of Biological Chemistry.

[12]  R. Berges,et al.  Effect of a new leuprorelin formulation on testosterone levels in patients with advanced prostate cancer , 2006, Current medical research and opinion.

[13]  A. Shevchenko,et al.  Huntingtin–HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease , 2006, The Journal of cell biology.

[14]  L. Ellerby,et al.  Ataxin-7 Can Export from the Nucleus via a Conserved Exportin-dependent Signal* , 2006, Journal of Biological Chemistry.

[15]  Walter Birchmeier,et al.  Balancing cell adhesion and Wnt signaling, the key role of β-catenin , 2006 .

[16]  E. Wanker,et al.  Interaction of huntingtin fragments with brain membranes – clues to early dysfunction in Huntington's disease , 2006, Journal of neurochemistry.

[17]  J. Sutcliffe,et al.  Selective deficits in the expression of striatal‐enriched mRNAs in Huntington's disease , 2006, Journal of neurochemistry.

[18]  A. Levine,et al.  p53 tumor suppressor protein regulates the levels of huntingtin gene expression , 2006, Oncogene.

[19]  J. Mallet,et al.  Lentiviral‐mediated gene transfer of brain‐derived neurotrophic factor is neuroprotective in a mouse model of neonatal excitotoxic challenge , 2006, Journal of neuroscience research.

[20]  C. Chiang,et al.  The General Transcription Machinery and General Cofactors , 2006, Critical reviews in biochemistry and molecular biology.

[21]  R. Snell,et al.  TATA-binding protein in neurodegenerative disease , 2005, Neuroscience.

[22]  Elena Cattaneo,et al.  Normal huntingtin function: an alternative approach to Huntington's disease , 2005, Nature Reviews Neuroscience.

[23]  Emmanuel Brouillet,et al.  Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment , 2005, Neurobiology of Disease.

[24]  W. H. Goldmann,et al.  Huntingtin Associates with Acidic Phospholipids at the Plasma Membrane* , 2005, Journal of Biological Chemistry.

[25]  S. Cregan,et al.  Inhibition of Metabotropic Glutamate Receptor Signaling by the Huntingtin-binding Protein Optineurin* , 2005, Journal of Biological Chemistry.

[26]  A. El-Husseini,et al.  Modulation of neuronal protein trafficking and function by palmitoylation , 2005, Current Opinion in Neurobiology.

[27]  M. Chesselet,et al.  Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing , 2005, Neurobiology of Disease.

[28]  Elena Cattaneo,et al.  Progressive loss of BDNF in a mouse model of Huntington's disease and rescue by BDNF delivery. , 2005, Pharmacological research.

[29]  I. Mills,et al.  Huntingtin interacting protein 1 modulates the transcriptional activity of nuclear hormone receptors , 2005, The Journal of cell biology.

[30]  S. Snyder,et al.  p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease , 2005, Neuron.

[31]  B. Chait,et al.  Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[32]  Yoshiyuki Matsuura,et al.  Structural basis for nuclear import complex dissociation by RanGTP , 2005, Nature.

[33]  A. Aguilera Cotranscriptional mRNP assembly: from the DNA to the nuclear pore. , 2005, Current opinion in cell biology.

[34]  Harry T Orr,et al.  A cell-based screen for modulators of ataxin-1 phosphorylation. , 2005, Human molecular genetics.

[35]  R. Margolis,et al.  Huntington's disease like-2: review and update. , 2005, Acta neurologica Taiwanica.

[36]  Holger Wesche,et al.  High throughput screening for protein kinase inhibitors. , 2005, Combinatorial chemistry & high throughput screening.

[37]  Fumiaki Tanaka,et al.  Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. , 2005, Brain : a journal of neurology.

[38]  A. Young,et al.  A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[39]  M. MacDonald,et al.  Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation , 2005, BMC Neuroscience.

[40]  Harry T Orr,et al.  RNA association and nucleocytoplasmic shuttling by ataxin-1 , 2005, Journal of Cell Science.

[41]  A. Hopkins,et al.  Navigating chemical space for biology and medicine , 2004, Nature.

[42]  U. Moens,et al.  What turns CREB on? , 2004, Cellular signalling.

[43]  Harry T Orr,et al.  Recovery from Polyglutamine-Induced Neurodegeneration in Conditional SCA1 Transgenic Mice , 2004, The Journal of Neuroscience.

[44]  M. Fainzilber,et al.  Integration of Retrograde Axonal and Nuclear Transport Mechanisms in Neurons: Implications for Therapeutics , 2004, The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry.

[45]  Ilya Bezprozvanny,et al.  Deranged neuronal calcium signaling and Huntington disease. , 2004, Biochemical and biophysical research communications.

[46]  C. Dargemont,et al.  Nuclear export of RNA , 2004, Biology of the cell.

[47]  H. Lehrach,et al.  A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. , 2004, Molecular cell.

[48]  P. Patterson,et al.  Activation of the IκB Kinase Complex and Nuclear Factor-κB Contributes to Mutant Huntingtin Neurotoxicity , 2004, The Journal of Neuroscience.

[49]  D. Borchelt,et al.  Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice. , 2004, Human molecular genetics.

[50]  Fabrice P Cordelières,et al.  Huntingtin Controls Neurotrophic Support and Survival of Neurons by Enhancing BDNF Vesicular Transport along Microtubules , 2004, Cell.

[51]  M. Hayden,et al.  Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity , 2004, Journal of Biological Chemistry.

[52]  Thorsten Schmidt,et al.  Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis , 2004, The Lancet Neurology.

[53]  J. Littleton,et al.  Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[54]  Fumiaki Tanaka,et al.  Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives , 2004, Journal of Molecular Medicine.

[55]  Christine Klein,et al.  Focal dystonia as a presenting sign of spinocerebellar ataxia 17 , 2004, Movement disorders : official journal of the Movement Disorder Society.

[56]  M. Hayden,et al.  Huntingtin Bodies Sequester Vesicle-Associated Proteins by a Polyproline-Dependent Interaction , 2004, The Journal of Neuroscience.

[57]  John D. Aitchison,et al.  Cell Cycle Regulated Transport Controlled by Alterations in the Nuclear Pore Complex , 2003, Cell.

[58]  M. Koltzenburg,et al.  Axoplasmic Importins Enable Retrograde Injury Signaling in Lesioned Nerve , 2003, Neuron.

[59]  P. Giannakakou,et al.  The importance of p53 location: nuclear or cytoplasmic zip code? , 2003, Drug resistance updates : reviews and commentaries in antimicrobial and anticancer chemotherapy.

[60]  V. Bolivar,et al.  Exploratory activity and fear conditioning abnormalities develop early in R6/2 Huntington's disease transgenic mice. , 2003, Behavioral neuroscience.

[61]  Leslie Michels Thompson,et al.  A Rapid Cellular FRET Assay of Polyglutamine Aggregation Identifies a Novel Inhibitor , 2003, Neuron.

[62]  G. Sobue [Development of therapeutics for spinal and bulbar muscular atrophy (SBMA)]. , 2003, Rinsho shinkeigaku = Clinical neurology.

[63]  Qiuheng Zhang,et al.  Identification and Characterization of a Ligand-regulated Nuclear Export Signal in Androgen Receptor* , 2003, Journal of Biological Chemistry.

[64]  Martin Drozda,et al.  Depletion of wild‐type huntingtin in mouse models of neurologic diseases , 2003, Journal of neurochemistry.

[65]  J. Roh,et al.  Huntingtin is degraded to small fragments by calpain after ischemic injury☆ , 2003, Experimental Neurology.

[66]  Andrea Crotti,et al.  Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes , 2003, Nature Genetics.

[67]  P. Chaudhary,et al.  Use of adeno-associated viral vector for delivery of small interfering RNA , 2003, Oncogene.

[68]  R. Truant,et al.  Huntingtin contains a highly conserved nuclear export signal. , 2003, Human molecular genetics.

[69]  Deborah L. Johnson,et al.  The TATA-Binding Protein as a Regulator of Cellular Transformation , 2003, Cell cycle.

[70]  Effat S. Emamian,et al.  Serine 776 of Ataxin-1 Is Critical for Polyglutamine-Induced Disease in SCA1 Transgenic Mice , 2003, Neuron.

[71]  L. Farrell,et al.  Mutant huntingtin increases nuclear corepressor function and enhances ligand-dependent nuclear hormone receptor activation , 2003, Molecular and Cellular Neuroscience.

[72]  M. Hayden,et al.  Nuclear Localization of a Non-caspase Truncation Product of Atrophin-1, with an Expanded Polyglutamine Repeat, Increases Cellular Toxicity* , 2003, The Journal of Biological Chemistry.

[73]  T. Littlewood,et al.  GLFG and FxFG Nucleoporins Bind to Overlapping Sites on Importin-β* , 2002, The Journal of Biological Chemistry.

[74]  S. Hersch,et al.  HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. , 2002, Human molecular genetics.

[75]  J. Gusella,et al.  The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor , 2002, BMC Neuroscience.

[76]  Richard I. Morimoto,et al.  Polyglutamine protein aggregates are dynamic , 2002, Nature Cell Biology.

[77]  M. Mancini,et al.  Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components , 2002, Nature Cell Biology.

[78]  G. Sobue,et al.  Testosterone Reduction Prevents Phenotypic Expression in a Transgenic Mouse Model of Spinal and Bulbar Muscular Atrophy , 2002, Neuron.

[79]  J. Olson,et al.  Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects. , 2002, Human molecular genetics.

[80]  J. Olson,et al.  Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. , 2002, Human molecular genetics.

[81]  M. Hetzer,et al.  The Ran GTPase as a marker of chromosome position in spindle formation and nuclear envelope assembly , 2002, Nature Cell Biology.

[82]  H. Paulson,et al.  Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis , 2002, Proceedings of the National Academy of Sciences of the United States of America.

[83]  D. Görlich,et al.  The permeability barrier of nuclear pore complexes appears to operate via hydrophobic exclusion , 2002, The EMBO journal.

[84]  K. Wilson,et al.  The nuclear envelope, lamins and nuclear assembly. , 2002, Current opinion in cell biology.

[85]  M. Hayden,et al.  Targeted disruption of Huntingtin-associated protein-1 (Hap1) results in postnatal death due to depressed feeding behavior. , 2002, Human molecular genetics.

[86]  J. Alberch,et al.  Neuroprotection by neurotrophins and GDNF family members in the excitotoxic model of Huntington’s disease , 2002, Brain Research Bulletin.

[87]  D. Housman,et al.  A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila , 2002, Nature Genetics.

[88]  J. Nevins,et al.  Huntingtin Is Present in the Nucleus, Interacts with the Transcriptional Corepressor C-terminal Binding Protein, and Represses Transcription* , 2002, The Journal of Biological Chemistry.

[89]  P. Silver,et al.  Protein and RNA export from the nucleus. , 2002, Developmental cell.

[90]  B. Henderson,et al.  ARM Domain-dependent Nuclear Import of Adenomatous Polyposis Coli Protein Is Stimulated by the B56α Subunit of Protein Phosphatase 2A* , 2001, The Journal of Biological Chemistry.

[91]  I. Macara Transport into and out of the Nucleus , 2001, Microbiology and Molecular Biology Reviews.

[92]  A. Silkov,et al.  A Composite Nuclear Export Signal in the TBP-associated Factor TAFII105* , 2001, The Journal of Biological Chemistry.

[93]  J. Darnell,et al.  Microarray Identification of FMRP-Associated Brain mRNAs and Altered mRNA Translational Profiles in Fragile X Syndrome , 2001, Cell.

[94]  B. Majello,et al.  Control of RNA polymerase II activity by dedicated CTD kinases and phosphatases. , 2001, Frontiers in bioscience : a journal and virtual library.

[95]  P. Marynen,et al.  NXF5, a novel member of the nuclear RNA export factor family, is lost in a male patient with a syndromic form of mental retardation , 2001, Current Biology.

[96]  P. Reddy,et al.  Polyglutamine-expanded Huntingtin Promotes Sensitization of N-Methyl-d-aspartate Receptors via Post-synaptic Density 95* , 2001, The Journal of Biological Chemistry.

[97]  Blair R. Leavitt,et al.  Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease , 2001, Science.

[98]  T. Littlewood,et al.  Molecular mechanism of translocation through nuclear pore complexes during nuclear protein import , 2001, FEBS letters.

[99]  T. Wada,et al.  The regulation of elongation by eukaryotic RNA polymerase II: a recent view. , 2001, Molecules and cells.

[100]  P Bork,et al.  Comparison of ARM and HEAT protein repeats. , 2001, Journal of molecular biology.

[101]  William I. Weis,et al.  The Structure of the β-Catenin/E-Cadherin Complex and the Molecular Basis of Diverse Ligand Recognition by β-Catenin , 2001, Cell.

[102]  D. Rigamonti,et al.  Huntingtin's Neuroprotective Activity Occurs via Inhibition of Procaspase-9 Processing* , 2001, The Journal of Biological Chemistry.

[103]  H. Lehrach,et al.  Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. , 2001, Molecular biology of the cell.

[104]  F. Gage,et al.  Identities of Sequestered Proteins in Aggregates from Cells with Induced Polyglutamine Expression , 2001, The Journal of cell biology.

[105]  C A Ross,et al.  Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity , 2001, Science.

[106]  K Ribbeck,et al.  Kinetic analysis of translocation through nuclear pore complexes , 2001, The EMBO journal.

[107]  C. Ross,et al.  Isolation of a 40-kDa Huntingtin-associated Protein* , 2001, The Journal of Biological Chemistry.

[108]  J. Hodgson,et al.  Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. , 2001, American journal of human genetics.

[109]  M. MacDonald,et al.  Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles. , 2000, Human molecular genetics.

[110]  B. Cullen,et al.  Adenomatous polyposis coli protein contains two nuclear export signals and shuttles between the nucleus and cytoplasm. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[111]  K. Arahata,et al.  Nuclear envelope proteins and associated diseases , 2000, Current opinion in neurology.

[112]  B. Henderson Nuclear-cytoplasmic shuttling of APC regulates β-catenin subcellular localization and turnover , 2000, Nature Cell Biology.

[113]  M. MacDonald,et al.  Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis. , 2000, Human molecular genetics.

[114]  Mariann Bienz,et al.  The APC tumour suppressor has a nuclear export function , 2000, Nature.

[115]  D. Housman,et al.  The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[116]  S. Wente Gatekeepers of the nucleus. , 2000, Science.

[117]  S. Pulst,et al.  A novel protein with RNA-binding motifs interacts with ataxin-2. , 2000, Human molecular genetics.

[118]  A. Hackam,et al.  Wild-Type Huntingtin Protects from Apoptosis Upstream of Caspase-3 , 2000, The Journal of Neuroscience.

[119]  H. Zoghbi,et al.  Fourteen and counting: unraveling trinucleotide repeat diseases. , 2000, Human molecular genetics.

[120]  René Hen,et al.  Reversal of Neuropathology and Motor Dysfunction in a Conditional Model of Huntington's Disease , 2000, Cell.

[121]  B. Chait,et al.  The Yeast Nuclear Pore Complex: Composition, Architecture, and Transport Mechanism , 2000 .

[122]  D. Andrews,et al.  Identification of the Endoplasmic Reticulum Targeting Signal in Vesicle-associated Membrane Proteins* , 1999, The Journal of Biological Chemistry.

[123]  T. Hope,et al.  An N‐terminal nuclear export signal is required for the nucleocytoplasmic shuttling of IκBα , 1999 .

[124]  B. Rayet,et al.  Aberrant rel/nfkb genes and activity in human cancer , 1999, Oncogene.

[125]  B. Cullen,et al.  The Human Tap Nuclear RNA Export Factor Contains a Novel Transportin-dependent Nuclear Localization Signal That Lacks Nuclear Export Signal Function* , 1999, The Journal of Biological Chemistry.

[126]  M. Azumano,et al.  Nuclear localization of transcription factor Sp1. , 1999, Nucleic acids symposium series.

[127]  M. MacDonald,et al.  Mutant Huntingtin Forms in Vivo Complexes with Distinct Context-Dependent Conformations of the Polyglutamine Segment , 1999, Neurobiology of Disease.

[128]  G. Suske The Sp-family of transcription factors. , 1999, Gene.

[129]  J. M. Boutell,et al.  Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. , 1999, Human molecular genetics.

[130]  D. Borchelt,et al.  Nuclear Accumulation of Truncated Atrophin-1 Fragments in a Transgenic Mouse Model of DRPLA , 1999, Neuron.

[131]  M. Nachury,et al.  The direction of transport through the nuclear pore can be inverted. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[132]  C. Ross,et al.  Nuclear Targeting of Mutant Huntingtin Increases Toxicity , 1999, Molecular and Cellular Neuroscience.

[133]  D. Housman,et al.  Evidence for a recruitment and sequestration mechanism in Huntington's disease. , 1999, Philosophical transactions of the Royal Society of London. Series B, Biological sciences.

[134]  Jones Al The localization and interactions of huntingtin , 1999 .

[135]  Claire-Anne Gutekunst,et al.  A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal Neurodegeneration , 1999, Neuron.

[136]  R. Myers,et al.  Impaired synaptic plasticity in mice carrying the Huntington's disease mutation. , 1999, Human molecular genetics.

[137]  G. Wahl,et al.  A leucine‐rich nuclear export signal in the p53 tetramerization domain: regulation of subcellular localization and p53 activity by NES masking , 1999, The EMBO journal.

[138]  R. Truant,et al.  Nuclear Import of Cdk/Cyclin Complexes: Identification of Distinct Mechanisms for Import of Cdk2/Cyclin E and Cdc2/Cyclin B1 , 1999, The Journal of cell biology.

[139]  K. Moulder,et al.  Generation of Neuronal Intranuclear Inclusions by Polyglutamine-GFP: Analysis of Inclusion Clearance and Toxicity as a Function of Polyglutamine Length , 1999, The Journal of Neuroscience.

[140]  L. Poellinger,et al.  Signal transduction in hypoxic cells: inducible nuclear translocation and recruitment of theCBP/p300 coactivator by the hypoxia‐induciblefactor‐1α , 1998, The EMBO journal.

[141]  Harry T Orr,et al.  Ataxin-1 Nuclear Localization and Aggregation Role in Polyglutamine-Induced Disease in SCA1 Transgenic Mice , 1998, Cell.

[142]  Steven Finkbeiner,et al.  Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear Inclusions , 1998, Cell.

[143]  M. MacDonald,et al.  Huntingtin interacts with a family of WW domain proteins. , 1998, Human molecular genetics.

[144]  G. Stein,et al.  Interrelationships of nuclear structure and transcriptional control: Functional consequences of being in the right place at the right time , 1998, Journal of cellular biochemistry.

[145]  M. Yanagida,et al.  Leptomycin B inhibition of signal-mediated nuclear export by direct binding to CRM1. , 1998, Experimental cell research.

[146]  M. MacDonald,et al.  Huntingtin: a single bait hooks many species , 1998, Current Opinion in Neurobiology.

[147]  G. Blobel,et al.  Transport routes through the nuclear pore complex. , 1998, Current opinion in cell biology.

[148]  M. Rout,et al.  Karyopherins and kissing cousins. , 1998, Trends in cell biology.

[149]  C. Ross,et al.  HAP1‐huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice , 1998, FEBS letters.

[150]  Dale E. Bredesen,et al.  Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract* , 1998, The Journal of Biological Chemistry.

[151]  B. Cullen,et al.  Identification and Functional Characterization of a Novel Nuclear Localization Signal Present in the Yeast Nab2 Poly(A)+ RNA Binding Protein , 1998, Molecular and Cellular Biology.

[152]  R. Albin,et al.  Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse , 1997, Cell.

[153]  P. Worley,et al.  Huntingtin-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin. , 1997, Human molecular genetics.

[154]  S. W. Davies,et al.  Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. , 1997, Science.

[155]  Minoru Yoshida,et al.  CRM1 Is an Export Receptor for Leucine-Rich Nuclear Export Signals , 1997, Cell.

[156]  R. Vallee,et al.  Fast transport and retrograde movement of huntingtin and HAP 1 in axons , 1997, Neuroreport.

[157]  Mark Turmaine,et al.  Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation , 1997, Cell.

[158]  K. Kinzler,et al.  Constitutive Transcriptional Activation by a β-Catenin-Tcf Complex in APC−/− Colon Carcinoma , 1997, Science.

[159]  H. Lehrach,et al.  HIP-I: a huntingtin interacting protein isolated by the yeast two-hybrid system. , 1997, Human molecular genetics.

[160]  S. W. Davies,et al.  Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice , 1996, Cell.

[161]  B. Cullen,et al.  A nuclear role for the Fragile X mental retardation protein. , 1996, The EMBO journal.

[162]  J. D. den Dunnen,et al.  Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation. , 1996, Human molecular genetics.

[163]  S. Snyder,et al.  Huntingtin-associated protein (HAP1): discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase. , 1996, Proceedings of the National Academy of Sciences of the United States of America.

[164]  S. Snyder,et al.  A huntingtin-associated protein enriched in brain with implications for pathology , 1995, Nature.

[165]  Peer Bork,et al.  HEAT repeats in the Huntington's disease protein , 1995, Nature Genetics.

[166]  Virginia E. Papaioannou,et al.  Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue , 1995, Nature Genetics.

[167]  Harry T Orr,et al.  SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat , 1995, Cell.

[168]  R. Carraway,et al.  Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons , 1995, Neuron.

[169]  F. Masiarz,et al.  Association of the APC gene product with beta-catenin. , 1993, Science.

[170]  K. Fenger,et al.  Trinucleotide repeat elongation in the Huntingtin gene in Huntington disease patients from 71 Danish families. , 1993, Human molecular genetics.

[171]  Manish S. Shah,et al.  A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes , 1993, Cell.

[172]  C. Ingles,et al.  Direct interaction between the transcriptional activation domain of human p53 and the TATA box-binding protein. , 1993, The Journal of biological chemistry.

[173]  J. Greenblatt Riding high on the TATA box , 1992, Nature.

[174]  K. Fischbeck,et al.  Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy , 1991, Nature.

[175]  R. Ferrante,et al.  Neuropathological Classification of Huntington's Disease , 1985, Journal of neuropathology and experimental neurology.

[176]  M. Kelly,et al.  SNARE complex regulation by phosphorylation , 2006, Cell Biochemistry and Biophysics.

[177]  L. Tora,et al.  Both normal and polyglutamine- expanded ataxin-7 are components of TFTC-type GCN5 histone acetyltransferase- containing complexes. , 2006, Biochemical Society symposium.

[178]  G. Coetzee,et al.  The androgen receptor: unlocking the secrets of its unique transactivation domain. , 2005, Vitamins and hormones.

[179]  R. Kopito,et al.  Impairment of the ubiquitin-proteasome system by protein aggregation. , 2001, Science.

[180]  H. Zoghbi,et al.  Glutamine repeats and neurodegeneration. , 2000, Annual review of neuroscience.

[181]  M. Bissell,et al.  Communication between the cell membrane and the nucleus: Role of protein compartmentalization , 1998, Journal of cellular biochemistry.

[182]  Y. Wang,et al.  Leptomycin B is an inhibitor of nuclear export: inhibition of nucleo-cytoplasmic translocation of the human immunodeficiency virus type 1 (HIV-1) Rev protein and Rev-dependent mRNA. , 1997, Chemistry & biology.

[183]  Murphy Ea,et al.  Familial polyposis coli. , 1980, Progress in medical genetics.