Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (162)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Lin, C.-H.
Right arrow Articles by Detloff, P. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Lin, C.-H.
Right arrow Articles by Detloff, P. J.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Human Molecular Genetics, 2001, Vol. 10, No. 2 137-144
© 2001 Oxford University Press

Neurological abnormalities in a knock-in mouse model of Huntington’s disease

Chin-Hsing Lin1, Sara Tallaksen-Greene2, Wei-Ming Chien1, Jamie A. Cearley1, Walker S. Jackson1, Andrew B. Crouse1, Songrong Ren1, Xiao-Jiang Li3, Roger L. Albin2,4 and Peter J. Detloff1,5,+

1Department of Biochemistry and Molecular Genetics and 5Department of Neurobiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA, 2Department of Neurology, University of Michigan and 4Geriatrics Research, Education and Clinical Center, Ann Arbor VAMC, Ann Arbor, MI 48104, USA and 3Department of Genetics, Emory University, Atlanta, GA 30322, USA

Mice representing precise genetic replicas of Huntington’s disease (HD) were made using gene targeting to replace the short CAG repeat of the mouse Huntington’s disease gene homolog (Hdh) with CAG repeats within the length range found to cause HD in humans. Mice with alleles of ~150 units in length exhibit late-onset behavioral and neuroanatomic abnormalities consistent with HD. These symptoms include a motor task deficit, gait abnormalities, reactive gliosis and the formation of neuronal intranuclear inclusions predominating in the striatum. This model differs from previously described Hdh knock-ins by its method of construction, longer repeat length and more severe phenotype. To our knowledge, this is the first knock-in mouse model of HD to show increased glial fibrillary acidic protein immunoreactivity in the striatum, suggesting that these mice have neuronal injury similar to that found early in the course of HD. These mice will serve as useful reagents in experiments designed to reveal the molecular nature of neuronal dysfunction underlying HD.

+ To whom correspondence should be addressed. Tel: +1 205 975 8157; Fax: +1 205 975 7928; Email: pdetloff@bmg.bhs.uab.edu


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 ScienceHome page
S. Subramaniam, K. M. Sixt, R. Barrow, and S. H. Snyder
Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity
Science, June 5, 2009; 324(5932): 1327 - 1330.
[Abstract] [Full Text] [PDF]

Home page
 DMMHome page
D. E. Ehrnhoefer, S. L. Butland, M. A. Pouladi, and M. R. Hayden
Mouse models of Huntington disease: variations on a theme
Dis. Model. Mech., March 1, 2009; 2(3-4): 123 - 129.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
L.-S. Her and L. S. B. Goldstein
Enhanced Sensitivity of Striatal Neurons to Axonal Transport Defects Induced by Mutant Huntingtin
J. Neurosci., December 10, 2008; 28(50): 13662 - 13672.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
S. Tydlacka, C.-E. Wang, X. Wang, S. Li, and X.-J. Li
Differential Activities of the Ubiquitin-Proteasome System in Neurons versus Glia May Account for the Preferential Accumulation of Misfolded Proteins in Neurons
J. Neurosci., December 3, 2008; 28(49): 13285 - 13295.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
C.-E. Wang, S. Tydlacka, A. L. Orr, S.-H. Yang, R. K. Graham, M. R. Hayden, S. Li, A. W.S. Chan, and X.-J. Li
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease
Hum. Mol. Genet., September 1, 2008; 17(17): 2738 - 2751.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
K. Watase, C. F. Barrett, T. Miyazaki, T. Ishiguro, K. Ishikawa, Y. Hu, T. Unno, Y. Sun, S. Kasai, M. Watanabe, et al.
Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels
PNAS, August 19, 2008; 105(33): 11987 - 11992.
[Abstract] [Full Text] [PDF]

Home page
 JEMHome page
M. Bjorkqvist, E. J. Wild, J. Thiele, A. Silvestroni, R. Andre, N. Lahiri, E. Raibon, R. V. Lee, C. L. Benn, D. Soulet, et al.
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease
J. Exp. Med., August 4, 2008; 205(8): 1869 - 1877.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
A. L. Orr, S. Huang, M. A. Roberts, J. C. Reed, S. Li, and X.-J. Li
Sex-dependent Effect of BAG1 in Ameliorating Motor Deficits of Huntington Disease Transgenic Mice
J. Biol. Chem., June 6, 2008; 283(23): 16027 - 16036.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
S.-Y. Chou, J.-Y. Weng, H.-L. Lai, F. Liao, S. H. Sun, P.-H. Tu, D. W. Dickson, and Y. Chern
Expanded-Polyglutamine Huntingtin Protein Suppresses the Secretion and Production of a Chemokine (CCL5/RANTES) by Astrocytes
J. Neurosci., March 26, 2008; 28(13): 3277 - 3290.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
J. Wang, C.-E. Wang, A. Orr, S. Tydlacka, S.-H. Li, and X.-J. Li
Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice
J. Cell Biol., March 24, 2008; 180(6): 1177 - 1189.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
A. L. Orr, S. Li, C.-E. Wang, H. Li, J. Wang, J. Rong, X. Xu, P. G. Mastroberardino, J. T. Greenamyre, and X.-J. Li
N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking
J. Neurosci., March 12, 2008; 28(11): 2783 - 2792.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
R. Gonitel, H. Moffitt, K. Sathasivam, B. Woodman, P. J. Detloff, R. L. M. Faull, and G. P. Bates
DNA instability in postmitotic neurons
PNAS, March 4, 2008; 105(9): 3467 - 3472.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
A. D. Strand, Z. C. Baquet, A. K. Aragaki, P. Holmans, L. Yang, C. Cleren, M. F. Beal, L. Jones, C. Kooperberg, J. M. Olson, et al.
Expression Profiling of Huntington's Disease Models Suggests That Brain-Derived Neurotrophic Factor Depletion Plays a Major Role in Striatal Degeneration
J. Neurosci., October 24, 2007; 27(43): 11758 - 11768.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. DiFiglia, M. Sena-Esteves, K. Chase, E. Sapp, E. Pfister, M. Sass, J. Yoder, P. Reeves, R. K. Pandey, K. G. Rajeev, et al.
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
PNAS, October 23, 2007; 104(43): 17204 - 17209.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. Y. Heng, S. J. Tallaksen-Greene, P. J. Detloff, and R. L. Albin
Longitudinal Evaluation of the Hdh(CAG)150 Knock-In Murine Model of Huntington's Disease
J. Neurosci., August 22, 2007; 27(34): 8989 - 8998.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A. Kuhn, D. R. Goldstein, A. Hodges, A. D. Strand, T. Sengstag, C. Kooperberg, K. Becanovic, M. A. Pouladi, K. Sathasivam, J.-H. J. Cha, et al.
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage
Hum. Mol. Genet., August 1, 2007; 16(15): 1845 - 1861.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M.-C. Chiang, H.-M. Chen, Y.-H. Lee, H.-H. Chang, Y.-C. Wu, B.-W. Soong, C.-M. Chen, Y.-R. Wu, C.-S. Liu, D.-M. Niu, et al.
Dysregulation of C/EBP{alpha} by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease
Hum. Mol. Genet., March 1, 2007; 16(5): 483 - 498.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
J. Cornett, L. Smith, M. Friedman, J.-Y. Shin, X.-J. Li, and S.-H. Li
Context-dependent Dysregulation of Transcription by Mutant Huntingtin
J. Biol. Chem., November 24, 2006; 281(47): 36198 - 36204.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A. Lloret, E. Dragileva, A. Teed, J. Espinola, E. Fossale, T. Gillis, E. Lopez, R. H. Myers, M. E. MacDonald, and V. C. Wheeler
Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice
Hum. Mol. Genet., June 15, 2006; 15(12): 2015 - 2024.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. M. Van Raamsdonk, W. T. Gibson, J. Pearson, Z. Murphy, G. Lu, B. R. Leavitt, and M. R. Hayden
Body weight is modulated by levels of full-length Huntingtin
Hum. Mol. Genet., May 1, 2006; 15(9): 1513 - 1523.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. Arango, S. Holbert, D. Zala, E. Brouillet, J. Pearson, E. Regulier, A. K. Thakur, P. Aebischer, R. Wetzel, N. Deglon, et al.
CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity.
J. Neurosci., April 26, 2006; 26(17): 4649 - 4659.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
Z. Yu, N. Dadgar, M. Albertelli, A. Scheller, R. L. Albin, D. M. Robins, and A. P. Lieberman
Abnormalities of Germ Cell Maturation and Sertoli Cell Cytoskeleton in Androgen Receptor 113 CAG Knock-In Mice Reveal Toxic Effects of the Mutant Protein
Am. J. Pathol., January 1, 2006; 168(1): 195 - 204.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
J.-Y. Shin, Z.-H. Fang, Z.-X. Yu, C.-E. Wang, S.-H. Li, and X.-J. Li
Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity
J. Cell Biol., December 19, 2005; 171(6): 1001 - 1012.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. M. Van Raamsdonk, Z. Murphy, E. J. Slow, B. R. Leavitt, and M. R. Hayden
Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease
Hum. Mol. Genet., December 15, 2005; 14(24): 3823 - 3835.
[Abstract] [Full Text] [PDF]

Home page
 Vet PatholHome page
C. J. Zeiss
Neuroanatomical Phenotyping in the Mouse: The Dopaminergic System
Vet. Pathol., November 1, 2005; 42(6): 753 - 773.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
A. Iwata, J. C. Christianson, M. Bucci, L. M. Ellerby, N. Nukina, L. S. Forno, and R. R. Kopito
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation
PNAS, September 13, 2005; 102(37): 13135 - 13140.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A. D. Strand, A. K. Aragaki, D. Shaw, T. Bird, J. Holton, C. Turner, S. J. Tapscott, S. J. Tabrizi, A. H. Schapira, C. Kooperberg, et al.
Gene expression in Huntington's disease skeletal muscle: a potential biomarker
Hum. Mol. Genet., July 1, 2005; 14(13): 1863 - 1876.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
S. C. Warby, E. Y. Chan, M. Metzler, L. Gan, R. R. Singaraja, S. F. Crocker, H. A. Robertson, and M. R. Hayden
Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo
Hum. Mol. Genet., June 1, 2005; 14(11): 1569 - 1577.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. M. Van Raamsdonk, J. Pearson, D. A. Rogers, N. Bissada, A. W. Vogl, M. R. Hayden, and B. R. Leavitt
Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease
Hum. Mol. Genet., May 15, 2005; 14(10): 1379 - 1392.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
J. M. Van Raamsdonk, J. Pearson, E. J. Slow, S. M. Hossain, B. R. Leavitt, and M. R. Hayden
Cognitive Dysfunction Precedes Neuropathology and Motor Abnormalities in the YAC128 Mouse Model of Huntington's Disease
J. Neurosci., April 20, 2005; 25(16): 4169 - 4180.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
S. Q. Harper, P. D. Staber, X. He, S. L. Eliason, I. H. Martins, Q. Mao, L. Yang, R. M. Kotin, H. L. Paulson, and B. L. Davidson
From the Cover: RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
PNAS, April 19, 2005; 102(16): 5820 - 5825.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
H. Adachi, M. Katsuno, M. Minamiyama, M. Waza, C. Sang, Y. Nakagomi, Y. Kobayashi, F. Tanaka, M. Doyu, A. Inukai, et al.
Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients
Brain, March 1, 2005; 128(3): 659 - 670.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
C. M. Everett and N. W. Wood
Trinucleotide repeats and neurodegenerative disease
Brain, November 1, 2004; 127(11): 2385 - 2405.
[Abstract] [Full Text] [PDF]

Home page
 NeuroscientistHome page
S.-H. Li and X.-J. Li
Huntington and its Role in Neuronal Degeneration
Neuroscientist, October 1, 2004; 10(5): 467 - 475.
[Abstract] [PDF]

Home page
 J. Neurosci.Home page
A. Khoshnan, J. Ko, E. E. Watkin, L. A. Paige, P. H. Reinhart, and P. H. Patterson
Activation of the I{kappa}B Kinase Complex and Nuclear Factor-{kappa}B Contributes to Mutant Huntingtin Neurotoxicity
J. Neurosci., September 15, 2004; 24(37): 7999 - 8008.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
G. Schilling, A. V. Savonenko, A. Klevytska, J. L. Morton, S. M. Tucker, M. Poirier, A. Gale, N. Chan, V. Gonzales, H. H. Slunt, et al.
Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice
Hum. Mol. Genet., August 1, 2004; 13(15): 1599 - 1610.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
Y. S. Choo, G. V.W. Johnson, M. MacDonald, P. J. Detloff, and M. Lesort
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
Hum. Mol. Genet., July 15, 2004; 13(14): 1407 - 1420.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
J. Gafni, E. Hermel, J. E. Young, C. L. Wellington, M. R. Hayden, and L. M. Ellerby
Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity: ACCUMULATION OF CALPAIN/CASPASE FRAGMENTS IN THE NUCLEUS
J. Biol. Chem., May 7, 2004; 279(19): 20211 - 20220.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
Q. Ruan, M. Lesort, M. E. MacDonald, and G. V.W. Johnson
Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway
Hum. Mol. Genet., April 1, 2004; 13(7): 669 - 681.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
L. Kennedy, E. Evans, C.-M. Chen, L. Craven, P. J. Detloff, M. Ennis, and P. F. Shelbourne
Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis
Hum. Mol. Genet., December 15, 2003; 12(24): 3359 - 3367.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
S. Gines, E. Ivanova, I.-S. Seong, C. A. Saura, and M. E. MacDonald
Enhanced Akt Signaling Is an Early Pro-survival Response That Reflects N-Methyl-D-aspartate Receptor Activation in Huntington's Disease Knock-in Striatal Cells
J. Biol. Chem., December 12, 2003; 278(50): 50514 - 50522.
[Abstract] [Full Text] [PDF]

Home page
 Sci SignalHome page
M. E. MacDonald
Huntingtin: Alive and Well and Working in Middle Management
Sci. Signal., November 4, 2003; 2003(207): pe48 - pe48.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
E. Regulier, Y. Trottier, V. Perrin, P. Aebischer, and N. Deglon
Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum
Hum. Mol. Genet., November 1, 2003; 12(21): 2827 - 2836.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
H. Zhou, F. Cao, Z. Wang, Z.-X. Yu, H.-P. Nguyen, J. Evans, S.-H. Li, and X.-J. Li
Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity
J. Cell Biol., October 13, 2003; 163(1): 109 - 118.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. Glynn, R. A. Bortnick, and A. J. Morton
Complexin II is essential for normal neurological function in mice
Hum. Mol. Genet., October 1, 2003; 12(19): 2431 - 2448.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
H. Li, T. Wyman, Z.-X. Yu, S.-H. Li, and X.-J. Li
Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release
Hum. Mol. Genet., August 15, 2003; 12(16): 2021 - 2030.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
E. J. Slow, J. van Raamsdonk, D. Rogers, S. H. Coleman, R. K. Graham, Y. Deng, R. Oh, N. Bissada, S. M. Hossain, Y.-Z. Yang, et al.
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
Hum. Mol. Genet., July 1, 2003; 12(13): 1555 - 1567.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. Xia, D. H. Lee, J. Taylor, M. Vandelft, and R. Truant
Huntingtin contains a highly conserved nuclear export signal
Hum. Mol. Genet., June 15, 2003; 12(12): 1393 - 1403.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
V. M. Miller, H. Xia, G. L. Marrs, C. M. Gouvion, G. Lee, B. L. Davidson, and H. L. Paulson
Allele-specific silencing of dominant disease genes
PNAS, June 10, 2003; 100(12): 7195 - 7200.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
F. Squitieri, C. Gellera, M. Cannella, C. Mariotti, G. Cislaghi, D. C. Rubinsztein, E. W. Almqvist, D. Turner, A.-C. Bachoud-Levi, S. A. Simpson, et al.
Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course
Brain, April 1, 2003; 126(4): 946 - 955.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
Z.-X. Yu, S.-H. Li, J. Evans, A. Pillarisetti, H. Li, and X.-J. Li
Mutant Huntingtin Causes Context-Dependent Neurodegeneration in Mice with Huntington's Disease
J. Neurosci., March 15, 2003; 23(6): 2193 - 2202.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
E. Fossale, V. C. Wheeler, V. Vrbanac, L.-A. Lebel, A. Teed, J. S. Mysore, J. F. Gusella, M. E. MacDonald, and F. Persichetti
Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice
Hum. Mol. Genet., September 15, 2002; 11(19): 2233 - 2241.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
L. B. Menalled, J. D. Sison, Y. Wu, M. Olivieri, X.-J. Li, H. Li, S. Zeitlin, and M.-F. Chesselet
Early Motor Dysfunction and Striosomal Distribution of Huntingtin Microaggregates in Huntington's Disease Knock-In Mice
J. Neurosci., September 15, 2002; 22(18): 8266 - 8276.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
R. Luthi-Carter, S. A. Hanson, A. D. Strand, D. A. Bergstrom, W. Chun, N. L. Peters, A. M. Woods, E. Y. Chan, C. Kooperberg, D. Krainc, et al.
Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain
Hum. Mol. Genet., August 15, 2002; 11(17): 1911 - 1926.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
J. Gafni and L. M. Ellerby
Calpain Activation in Huntington's Disease
J. Neurosci., June 15, 2002; 22(12): 4842 - 4849.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
G. A. Garden, R. T. Libby, Y.-H. Fu, Y. Kinoshita, J. Huang, D. E. Possin, A. C. Smith, R. A. Martinez, G. C. Fine, S. K. Grote, et al.
Polyglutamine-Expanded Ataxin-7 Promotes Non-Cell-Autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice
J. Neurosci., June 15, 2002; 22(12): 4897 - 4905.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
L. P. de Almeida, C. A. Ross, D. Zala, P. Aebischer, and N. Deglon
Lentiviral-Mediated Delivery of Mutant Huntingtin in the Striatum of Rats Induces a Selective Neuropathology Modulated by Polyglutamine Repeat Size, Huntingtin Expression Levels, and Protein Length
J. Neurosci., May 1, 2002; 22(9): 3473 - 3483.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
Z.-X. Yu, S.-H. Li, H.-P. Nguyen, and X.-J. Li
Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice
Hum. Mol. Genet., April 15, 2002; 11(8): 905 - 914.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
V. C. Wheeler, C.-A. Gutekunst, V. Vrbanac, L.-A. Lebel, G. Schilling, S. Hersch, R. M. Friedlander, J. F. Gusella, J.-P. Vonsattel, D. R. Borchelt, et al.
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
Hum. Mol. Genet., March 1, 2002; 11(6): 633 - 640.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
S.-H. Li, A. L. Cheng, H. Zhou, S. Lam, M. Rao, H. Li, and X.-J. Li
Interaction of Huntington Disease Protein with Transcriptional Activator Sp1
Mol. Cell. Biol., March 1, 2002; 22(5): 1277 - 1287.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. M. Koeller, M. Woontner, L. S. Crnic, B. Kleinschmidt-DeMasters, J. Stephens, E. L. Hunt, and S. I. Goodman
Biochemical, pathologic and behavioral analysis of a mouse model of glutaric acidemia type I
Hum. Mol. Genet., February 1, 2002; 11(4): 347 - 357.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
G. A. Laforet, E. Sapp, K. Chase, C. McIntyre, F. M. Boyce, M. Campbell, B. A. Cadigan, L. Warzecki, D. A. Tagle, P. H. Reddy, et al.
Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's Disease
J. Neurosci., December 1, 2001; 21(23): 9112 - 9123.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
W. Auerbach, M. S. Hurlbert, P. Hilditch-Maguire, Y. Z. Wadghiri, V. C. Wheeler, S. I. Cohen, A. L. Joyner, M. E. MacDonald, and D. H. Turnbull
The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin
Hum. Mol. Genet., October 1, 2001; 10(22): 2515 - 2523.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
G. Yvert, K. S. Lindenberg, D. Devys, D. Helmlinger, G. B. Landwehrmeyer, and J.-L. Mandel
SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types
Hum. Mol. Genet., August 1, 2001; 10(16): 1679 - 1692.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
H. T. Orr
Beyond the Qs in the polyglutamine diseases
Genes & Dev., April 15, 2001; 15(8): 925 - 932.
[Full Text]

Home page
 JCBHome page
W. Chun, M. Lesort, J. Tucholski, C. A. Ross, and G. V.W. Johnson
Tissue Transglutaminase Does Not Contribute to the Formation of Mutant Huntingtin Aggregates
J. Cell Biol., April 2, 2001; 153(1): 25 - 34.
[Abstract] [Full Text] [PDF]


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.