Access the full text.
Sign up today, get DeepDyve free for 14 days.
Y. Li, Z. Li (2000)
[Mitochondria and apoptosis].Zhonghua yu fang yi xue za zhi [Chinese journal of preventive medicine], 34 3
(1998)
Length of the protein and polyglutamine tract influence localization and frequency of intracellular aggregates of huntingtin
D. Vistica, P. Skehan, D. Scudiero, A. Monks, Angela Pittman, M. Boyd (1991)
Tetrazolium-based assays for cellular viability: a critical examination of selected parameters affecting formazan production.Cancer research, 51 10
Jared Ordway, S. Tallaksen-Greene, C. Gutekunst, E. Bernstein, J. Cearley, H. Wiener, L. Dure, R. Lindsey, S. Hersch, R. Jope, R. Albin, P. Detloff (1997)
Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the MouseCell, 91
D. Newmeyer, D. Forbes (1988)
Nuclear import can be separated into distinct steps in vitro: Nuclear pore binding and translocationCell, 52
I. Klement, P. Skinner, M. Kaytor, H. Yi, S. Hersch, H. Clark, H. Zoghbi, H. Orr (1998)
Ataxin-1 Nuclear Localization and Aggregation Role in Polyglutamine-Induced Disease in SCA1 Transgenic MiceCell, 95
Xiao-Jiang Li, Shihua Li, A. Sharp, F. Nucifora, Gabriele Schilling, A. Lanahan, P. Worley, S. Snyder, C. Ross (1995)
A huntingtin-associated protein enriched in brain with implications for pathologyNature, 378
S. Igarashi, R. Koide, T. Shimohata, M. Yamada, Y. Hayashi, H. Takano, H. Date, M. Oyake, Toshiya Sato, A. Sato, S. Egawa, T. Ikeuchi, Hajime Tanaka, R. Nakano, Keiko Tanaka, I. Hozumi, T. Inuzuka, H. Takahashi, S. Tsuji (1998)
Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretchNature Genetics, 18
M. Difiglia, E. Sapp, K. Chase, S. Davies, G. Bates, J. Vonsattel, N. Aronin (1997)
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.Science, 277 5334
(1986)
Nucleocytoplasmic Transport
D. Tait, M. Riccio, A. Sittler, E. Scherzinger, S. Santi, A. Ognibene, N. Maraldi, H. Lehrach, E. Wanker (1998)
Ataxin-3 is transported into the nucleus and associates with the nuclear matrix.Human molecular genetics, 7 6
J. Carmichael, W. Degraff, A. Gazdar, J. Minna, James Mitchell (1987)
Evaluation of a tetrazolium-based semiautomated colorimetric assay: assessment of chemosensitivity testing.Cancer research, 47 4
M. Becher, J. Kotzuk, A. Sharp, S. Davies, G. Bates, D. Price, C. Ross (1998)
Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat LengthNeurobiology of Disease, 4
G. Stevanin, Y. Trottier, G. Cancel, A. Durr, Gilles David, O. Didierjean, K. Bürk, G. Imbert, F. Saudou, Myriem Abada-Bendib, I. Gourfinkel‐An, A. Benomar, N. Abbas, T. Klockgether, D. Grid, Y. Agid, J. Mandel, A. Brice (1996)
Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias.Human molecular genetics, 5 12
J. Warrick, H. Paulson, G. Gray-Board, Q. Bui, K. Fischbeck, R. Pittman, N. Bonini (1998)
Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in DrosophilaCell, 93
W. Wen, Judy Meinkotht, R. Tsien, Susan Taylor (1995)
Identification of a signal for rapid export of proteins from the nucleusCell, 82
H. Siomi, G. Dreyfuss (1995)
A nuclear localization domain in the hnRNP A1 proteinThe Journal of Cell Biology, 129
(1999)
Kennedy’s disease: Caspase cleavage of the androgen receptor is a crucial event in cytotoxicity
Y. Goldberg, M. Kalchman, M. Metzler, J. Nasir, J. Zeisler, R. Graham, H. Koide, J. O'kusky, A. Sharp, C. Ross, F. Jirik, M. Hayden (1996)
Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the human Huntington disease transcript.Human molecular genetics, 5 2
I. Gourfinkel‐An, G. Cancel, C. Duyckaerts, B. Faucheux, J. Hauw, Y. Trottier, A. Brice, Y. Agid, E. Hirsch (1998)
Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onsetNeuroReport, 9
C. Cummings, M. Mancini, B. Antalffy, D. DeFranco, H. Orr, H. Zoghbi (1998)
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1Nature Genetics, 19
C. Wellington, L. Ellerby, A. Hackam, R. Margolis, M. Trifiro, R. Singaraja, K. McCutcheon, G. Salvesen, S. Propp, M. Bromm, Kathleen Rowland, Taiqi Zhang, D. Rasper, Sophie Roy, N. Thornberry, L. Pinsky, A. Kakizuka, C. Ross, D. Nicholson, D. Bredesen, M. Hayden (1998)
Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract*The Journal of Biological Chemistry, 273
D. Merry, Y. Kobayashi, C. Bailey, A. Taye, K. Fischbeck (1998)
Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy.Human molecular genetics, 7 4
T. Miyashita, Y. Okamura-Oho, Yasuyuki Mito, S. Nagafuchi, M. Yamada (1997)
Dentatorubral Pallidoluysian Atrophy (DRPLA) Protein Is Cleaved by Caspase-3 during Apoptosis*The Journal of Biological Chemistry, 272
J. Cooper, G. Schilling, M. Peters, W. Herring, A. Sharp, Z. Kaminsky, J. Masone, F. Khan, M. Delanoy, D. Borchelt, V. Dawson, T. Dawson, C. Ross (1998)
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.Human molecular genetics, 7 5
M. Holmberg, C. Duyckaerts, A. Durr, G. Cancel, I. Gourfinkel‐An, P. Damier, B. Faucheux, Y. Trottier, E. Hirsch, Y. Agid, A. Brice (1998)
Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions.Human molecular genetics, 7 5
Y. Goldberg, D. Nicholson, D. Rasper, M. Kalchman, H. Koide, R. Graham, M. Bromm, P. Kazemi-Esfarjani, N. Thornberry, J. Vaillancourt, M. Hayden (1996)
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tractNature Genetics, 13
A. Hackam, C. Wellington, M. Hayden (1998)
The fatal attraction of polyglutamine‐containing proteinsClinical Genetics, 53
Anne Johnson, R. Terry (1974)
STUDIES ON BRAIN BIOPSIES OF PATIENTS WITH HUNTINGTON'S CHOREAJournal of Neuropathology and Experimental Neurology, 33
(1994)
Nucleic Acids Research Human Molecular Genetics
A. Lunkes, J. Mandel (1997)
Polyglutamines, nuclear inclusions and neurodegenerationNature Medicine, 3
P. Skinner, B. Koshy, C. Cummings, I. Klement, K. Helin, A. Servadio, H. Zoghbi, H. Orr (1997)
Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structuresNature, 389
H. Paulson, Matthew Perez, Y. Trottier, J. Trojanowski, S. Subramony, S. Das, P. Vig, J. Mandel, K. Fischbeck, R. Pittman (1997)
Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3Neuron, 19
S. Davies, M. Turmaine, Barbara Cozens, M. Difiglia, A. Sharp, C. Ross, E. Scherzinger, E. Wanker, L. Mangiarini, G. Bates (1997)
Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD MutationCell, 90
M. Kalchman, H. Koide, K. McCutcheon, R. Graham, K. Nichol, K. Nishiyama, P. Kazemi-Esfarjani, F. Lynn, C. Wellington, M. Metzler, Y. Goldberg, I. Kanazawa, R. Gietz, M. Hayden (1997)
HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brainNature Genetics, 16
A. Hackam, R. Singaraja, C. Wellington, M. Metzler, K. McCutcheon, Taiqi Zhang, M. Kalchman, M. Hayden (1998)
The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular ToxicityThe Journal of Cell Biology, 141
M. Schmidt-Zachmann, C. Dargemont, L. Kühn, E. Nigg (1993)
Nuclear export of proteins: The role of nuclear retentionCell, 74
T. Mosmann (1983)
Rapid colorimetric assay for cellular growth and survival: application to proliferation and cytotoxicity assays.Journal of immunological methods, 65 1-2
S. Andrew, Y. Goldberg, M. Hayden (1997)
Rethinking genotype and phenotype correlations in polyglutamine expansion disorders.Human molecular genetics, 6 12
J. Burke, J. Enghild, Margaret Martin, Y. Jou, R. Myers, A. Roses, J. Vance, W. Strittmatter (1996)
Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDHNature Medicine, 2
W. Couldwell, D. Hinton, Shi-kun He, Thomas Chen, Ibrahim Sebat, M. Weiss, R. Law (1994)
Protein kinase C inhibitors induce apoptosis in human malignant glioma cell linesFEBS Letters, 345
R. Butler, P. Leigh, M. McPhaul, J. Gallo (1998)
Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy.Human molecular genetics, 7 1
E. Sapp, Cordula Schwarz, K. Chase, P. Bhide, A. Young, Jr Penney, J. Vonsattel, N. Aronin, M. Difiglia (1997)
Huntingtin localization in brains of normal and Huntington's disease patientsAnnals of Neurology, 42
M. Kozak (1997)
Recognition of AUG and alternative initiator codons is augmented by G in position +4 but is not generally affected by the nucleotides in positions +5 and +6The EMBO Journal, 16
Y. Trottier, Y. Lutz, G. Stevanin, G. Imbert, D. Devys, G. Cancel, F. Saudou, C. Weber, Gilles David, L. Tora, Y. Agid, A. Brice, J. Mandel (1995)
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxiasNature, 378
C. Ross (1997)
Intranuclear Neuronal Inclusions: A Common Pathogenic Mechanism for Glutamine-Repeat Neurodegenerative Diseases?Neuron, 19
A. Lunkes, J. Mandel (1998)
A cellular model that recapitulates major pathogenic steps of Huntington's disease.Human molecular genetics, 7 9
A. Matilla, B. Koshy, C. Cummings, T. Isobe, H. Orr, H. Zoghbi (1997)
The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1Nature, 389
F. Saudou, S. Finkbeiner, D. Devys, M. Greenberg (1998)
Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear InclusionsCell, 95
Shihua Li, Xiao-Jiang Li (1998)
Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats.Human molecular genetics, 7 5
A unifying feature of the CAG expansion diseases is the formation of intracellular aggregates composed of the mutant polyglutamine-expanded protein. Despite the presence of aggregates in affected patients, the precise relationship between aggregates and disease pathogenesis is unresolved. Results from in vivo and in vitro studies of mutant huntingtin have lead to the hypothesis that nuclear localization of aggregates is critical for the pathology of Huntington's disease (HD). We tested this hypothesis using a 293T cell culture model system that compared the frequency and toxicity of cytoplasmic and nuclear huntingtin aggregates. We first assessed the mode of nuclear transport of N-terminal fragments of huntingtin, and show that the predicted endogenous NLS is not functional, providing data in support of passive nuclear transport. This result suggests that proteolysis is a necessary step for nuclear entry of huntingtin. Additionally, insertion of nuclear import or export sequences into huntingtin fragments containing 548 or 151 amino acids was used to reverse the normal localization of these proteins. Changing the subcellular localization of the fragments did not influence their total aggregate frequency. There were also no significant differences in toxicity associated with the presence of nuclear compared with cytoplasmic aggregates. The findings of nuclear and cytoplasmic aggregates in affected brains, together with these in vitro data, support the nucleus and cytosol as subcellular sites for pathogenesis in HD. © 1999 Oxford University Press « Previous | Next Article » Table of Contents This Article Hum. Mol. Genet. (1999) 8 (1): 25-33. doi: 10.1093/hmg/8.1.25 » Abstract Free Full Text (HTML) Free Full Text (PDF) Free Classifications Article Services Article metrics Alert me when cited Alert me if corrected Find similar articles Similar articles in Web of Science Similar articles in PubMed Add to my archive Download citation Request Permissions Citing Articles Load citing article information Citing articles via CrossRef Citing articles via Scopus Citing articles via Web of Science Citing articles via Google Scholar Google Scholar Articles by Hackam, A. S. Articles by Hayden, M. R. Search for related content PubMed PubMed citation Articles by Hackam, A. S. Articles by Singaraja, R. Articles by Zhang, T. Articles by Gan, L. Articles by Hayden, M. R. Related Content Load related web page information Share Email this article CiteULike Delicious Facebook Google+ Mendeley Twitter What's this? Search this journal: Advanced » Current Issue November 15, 2015 24 (22) Alert me to new issues The Journal About this journal Rights & Permissions Dispatch date of the next issue This journal is a member of the Committee on Publication Ethics (COPE) We are mobile – find out more Journals Career Network Impact factor: 6.393 5-Yr impact factor: 6.850 Executive Editors Professor Kay Davies Professor Anthony Wynshaw-Boris Professor Joel Hirschhorn Dr Jeffrey Barrett View full editorial board For Authors Instructions to authors Online submission Submit Now! Self-archiving policy Open access options for authors - visit Oxford Open This journal enables compliance with the NIH Public Access Policy Alerting Services Email table of contents Email Advance Access CiteTrack XML RSS feed Corporate Services Advertising sales Reprints Supplements var taxonomies = ("SCI01140"); Most Most Read Genetics of obesity and the prediction of risk for health Non-coding RNA Telomerase and cancer Ion channel diseases Down syndrome--recent progress and future prospects » View all Most Read articles Most Cited The DNA methyltransferases of mammals Nonsense-Mediated mRNA Decay in Health and Disease Mutation of human short tandem repeats Prediction of deleterious human alleles Isolation of a Candidate Human Telomerase Catalytic Subunit Gene, Which Reveals Complex Splicing Patterns in Different Cell Types » View all Most Cited articles Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department. Online ISSN 1460-2083 - Print ISSN 0964-6906 Copyright © 2015 Oxford University Press Oxford Journals Oxford University Press Site Map Privacy Policy Cookie Policy Legal Notices Frequently Asked Questions Other Oxford University Press sites: Oxford University Press Oxford Journals China Oxford Journals Japan Academic & Professional books Children's & Schools Books Dictionaries & Reference Dictionary of National Biography Digital Reference English Language Teaching Higher Education Textbooks International Education Unit Law Medicine Music Online Products & Publishing Oxford Bibliographies Online Oxford Dictionaries Online Oxford English Dictionary Oxford Language Dictionaries Online Oxford Scholarship Online Reference Rights and Permissions Resources for Retailers & Wholesalers Resources for the Healthcare Industry Very Short Introductions World's Classics function fnc_onDomLoaded() { var query_context = getQueryContext(); PF_initOIUnderbar(query_context,":QS:default","","JRN"); PF_insertOIUnderbar(0); }; if (window.addEventListener) { window.addEventListener('load', fnc_onDomLoaded, false); } else if (window.attachEvent) { window.attachEvent('onload', fnc_onDomLoaded); } var gaJsHost = (("https:" == document.location.protocol) ? "https://ssl." : "http://www."); document.write(unescape("%3Cscript src='" + gaJsHost + "google-analytics.com/ga.js' type='text/javascript'%3E%3C/script%3E")); try { var pageTracker = _gat._getTracker("UA-189672-16"); pageTracker._setDomainName(".oxfordjournals.org"); pageTracker._trackPageview(); } catch(err) {}
Human Molecular Genetics – Oxford University Press
Published: Jan 1, 1999
Read and print from thousands of top scholarly journals.
Already have an account? Log in
Bookmark this article. You can see your Bookmarks on your DeepDyve Library.
To save an article, log in first, or sign up for a DeepDyve account if you don’t already have one.
Copy and paste the desired citation format or use the link below to download a file formatted for EndNote
Access the full text.
Sign up today, get DeepDyve free for 14 days.
All DeepDyve websites use cookies to improve your online experience. They were placed on your computer when you launched this website. You can change your cookie settings through your browser.