Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.


Science 15 February 1985:
Vol. 227. no. 4688, pp. 770 - 773
DOI: 10.1126/science.3155875

Articles

Science, Vol 227, Issue 4688, 770-773
Copyright © 1985 by American Association for the Advancement of Science


articles

Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease

GA Graveland, RS Williams, and M DiFiglia

Golgi impregnations of neostriatum from deceased Huntington's disease patients and controls were examined. In all cases of Huntington's disease the morphology of dendrites of medium-sized spiny neurons was markedly altered by the appearance of recurved endings and appendages, a decrease or increase in the density of spines, and abnormalities in the size and shape of spines. Pathological changes were rarely observed in medium-sized and large aspiny neostriatal neurons. The findings provide evidence for simultaneous degeneration and growth of spiny neurons in Huntington's disease and support the view that a specific population of neostriatal neurons is selectively involved in its pathogenesis.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Selective neuronal requirement for huntingtin in the developing zebrafish.
T. L. Henshall, B. Tucker, A. L. Lumsden, S. Nornes, M. T. Lardelli, and R. I. Richards (2009)
Hum. Mol. Genet. 18, 4830-4842
   Abstract »    Full Text »    PDF »
Brain-specific Proteins Decline in the Cerebrospinal Fluid of Humans with Huntington Disease.
Q. Fang, A. Strand, W. Law, V. M. Faca, M. P. Fitzgibbon, N. Hamel, B. Houle, X. Liu, D. H. May, G. Poschmann, et al. (2009)
Mol. Cell. Proteomics 8, 451-466
   Abstract »    Full Text »    PDF »
Differential Susceptibility to Excitotoxic Stress in YAC128 Mouse Models of Huntington Disease between Initiation and Progression of Disease.
R. K. Graham, M. A. Pouladi, P. Joshi, G. Lu, Y. Deng, N.-P. Wu, B. E. Figueroa, M. Metzler, V. M. Andre, E. J. Slow, et al. (2009)
J. Neurosci. 29, 2193-2204
   Abstract »    Full Text »    PDF »
Histone Deacetylase Inhibition Modulates Kynurenine Pathway Activation in Yeast, Microglia, and Mice Expressing a Mutant Huntingtin Fragment.
F. Giorgini, T. Moller, W. Kwan, D. Zwilling, J. L. Wacker, S. Hong, L.-C. L. Tsai, C. S. Cheah, R. Schwarcz, P. Guidetti, et al. (2008)
J. Biol. Chem. 283, 7390-7400
   Abstract »    Full Text »    PDF »
Huntingtin-deficient zebrafish exhibit defects in iron utilization and development.
A. L. Lumsden, T. L. Henshall, S. Dayan, M. T. Lardelli, and R. I. Richards (2007)
Hum. Mol. Genet. 16, 1905-1920
   Abstract »    Full Text »    PDF »
Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain.
P. F. Shelbourne, C. Keller-McGandy, W. L. Bi, S.-R. Yoon, L. Dubeau, N. J. Veitch, J. P. Vonsattel, N. S. Wexler, The US-Venezuela Collaborative Research Group, N. Arnheim, et al. (2007)
Hum. Mol. Genet. 16, 1133-1142
   Abstract »    Full Text »    PDF »
Brain-Derived Neurotrophic Factor Restores Synaptic Plasticity in a Knock-In Mouse Model of Huntington's Disease.
G. Lynch, E. A. Kramar, C. S. Rex, Y. Jia, D. Chappas, C. M. Gall, and D. A. Simmons (2007)
J. Neurosci. 27, 4424-4434
   Abstract »    Full Text »    PDF »
Huntingtin-associated protein 1 (HAP1) interacts with androgen receptor (AR) and suppresses SBMA-mutant-AR-induced apoptosis.
Y. Takeshita, R. Fujinaga, C. Zhao, A. Yanai, and K. Shinoda (2006)
Hum. Mol. Genet. 15, 2298-2312
   Abstract »    Full Text »    PDF »
Flanking sequences profoundly alter polyglutamine toxicity in yeast.
M. L. Duennwald, S. Jagadish, P. J. Muchowski, and S. Lindquist (2006)
PNAS 103, 11045-11050
   Abstract »    Full Text »    PDF »
Expression and Characterization of Full-length Human Huntingtin, an Elongated HEAT Repeat Protein.
W. Li, L. C. Serpell, W. J. Carter, D. C. Rubinsztein, and J. A. Huntington (2006)
J. Biol. Chem. 281, 15916-15922
   Abstract »    Full Text »    PDF »
Altered Cortical Glutamate Receptor Function in the R6/2 Model of Huntington's Disease.
V. M. Andre, C. Cepeda, A. Venegas, Y. Gomez, and M. S. Levine (2006)
J Neurophysiol 95, 2108-2119
   Abstract »    Full Text »    PDF »
Polyglutamine expansion causes neurodegeneration by altering the neuronal differentiation program.
G. Abou-Sleymane, F. Chalmel, D. Helmlinger, A. Lardenois, C. Thibault, C. Weber, K. Merienne, J.-L. Mandel, O. Poch, D. Devys, et al. (2006)
Hum. Mol. Genet. 15, 691-703
   Abstract »    Full Text »    PDF »
The striatofugal fiber system in primates: A reevaluation of its organization based on single-axon tracing studies.
M. Levesque and A. Parent (2005)
PNAS 102, 11888-11893
   Abstract »    Full Text »    PDF »
Progression of structural neuropathology in preclinical Huntington's disease: a tensor based morphometry study.
C M Kipps, A J Duggins, N Mahant, L Gomes, J Ashburner, and E A McCusker (2005)
J. Neurol. Neurosurg. Psychiatry 76, 650-655
   Abstract »    Full Text »    PDF »
N-methyl-D-aspartate Receptor Subtypes: Multiple Roles in Excitotoxicity and Neurological Disease.
E. A. Waxman and D. R. Lynch (2005)
Neuroscientist 11, 37-49
   Abstract »    PDF »
Protection from mitochondrial complex II inhibition in vitro and in vivo by Nrf2-mediated transcription.
M. J. Calkins, R. J. Jakel, D. A. Johnson, K. Chan, Y. W. Kan, and J. A. Johnson (2005)
PNAS 102, 244-249
   Abstract »    Full Text »    PDF »
Huntington and its Role in Neuronal Degeneration.
S.-H. Li and X.-J. Li (2004)
Neuroscientist 10, 467-475
   Abstract »    PDF »
Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity: ACCUMULATION OF CALPAIN/CASPASE FRAGMENTS IN THE NUCLEUS.
J. Gafni, E. Hermel, J. E. Young, C. L. Wellington, M. R. Hayden, and L. M. Ellerby (2004)
J. Biol. Chem. 279, 20211-20220
   Abstract »    Full Text »    PDF »
Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis.
L. Kennedy, E. Evans, C.-M. Chen, L. Craven, P. J. Detloff, M. Ennis, and P. F. Shelbourne (2003)
Hum. Mol. Genet. 12, 3359-3367
   Abstract »    Full Text »    PDF »
Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease.
E. Y.W. Chan, R. Luthi-Carter, A. Strand, S. M. Solano, S. A. Hanson, M. M. DeJohn, C. Kooperberg, K. O. Chase, M. DiFiglia, A. B. Young, et al. (2002)
Hum. Mol. Genet. 11, 1939-1951
   Abstract »    Full Text »    PDF »
Characterization of a Brain-enriched Chaperone, MRJ, That Inhibits Huntingtin Aggregation and Toxicity Independently.
J.-Z. Chuang, H. Zhou, M. Zhu, S.-H. Li, X.-J. Li, and C.-H. Sung (2002)
J. Biol. Chem. 277, 19831-19838
   Abstract »    Full Text »    PDF »
Single-Cell Antisense RNA Amplification and Microarray Analysis as a Tool for Studying Neurological Degeneration and Restoration.
M. B. Kelz, G. W. Dent, S. Therianos, P. G. Marciano, T. K. McIntosh, P. D. Coleman, and J. H. Eberwine (2002)
Sci. Aging Knowl. Environ. 2002, re1-1
   Abstract »    Full Text »    PDF »
Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's Disease.
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. (2001)
J. Neurosci. 21, 9112-9123
   Abstract »    Full Text »    PDF »
Huntingtin Aggregate-Associated Axonal Degeneration is an Early Pathological Event in Huntington's Disease Mice.
H. Li, S.-H. Li, Z.-X. Yu, P. Shelbourne, and X.-J. Li (2001)
J. Neurosci. 21, 8473-8481
   Abstract »    Full Text »    PDF »
Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?.
L. Kennedy and P. F. Shelbourne (2000)
Hum. Mol. Genet. 9, 2539-2544
   Abstract »    Full Text »    PDF »
Huntington's disease progression: PET and clinical observations.
T. C. Andrews, R. A. Weeks, N. Turjanski, R. N. Gunn, L. H. A. Watkins, B. Sahakian, J. R. Hodges, A. E. Rosser, N. W. Wood, and D. J. Brooks (1999)
Brain 122, 2353-2363
   Abstract »    Full Text »    PDF »
Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity.
O. Hansson, A. Petersen, M. Leist, P. Nicotera, R. F. Castilho, and P. Brundin (1999)
PNAS 96, 8727-8732
   Abstract »    Full Text »    PDF »
Cellular Defects and Altered Gene Expression in PC12 Cells Stably Expressing Mutant Huntingtin.
S.-H. Li, A. L. Cheng, H. Li, and X.-J. Li (1999)
J. Neurosci. 19, 5159-5172
   Abstract »    Full Text »    PDF »
Nuclear and Neuropil Aggregates in Huntington's Disease: Relationship to Neuropathology.
C.-A. Gutekunst, S.-H. Li, H. Yi, J. S. Mulroy, S. Kuemmerle, R. Jones, D. Rye, R. J. Ferrante, S. M. Hersch, and X.-J. Li (1999)
J. Neurosci. 19, 2522-2534
   Abstract »    Full Text »    PDF »
Regulation of F-Actin Stability in Dendritic Spines by Glutamate Receptors and Calcineurin.
S. Halpain, A. Hipolito, and L. Saffer (1998)
J. Neurosci. 18, 9835-9844
   Abstract »    Full Text »    PDF »
Heterogeneous Topographic and Cellular Distribution of Huntingtin Expression in the Normal Human Neostriatum.
R. J. Ferrante, C.-A. Gutekunst, F. Persichetti, S. M. McNeil, N. W. Kowall, J. F. Gusella, M. E. MacDonald, M. F. Beal, and S. M. Hersch (1997)
J. Neurosci. 17, 3052-3063
   Abstract »    Full Text »    PDF »
Plastic Neuronal Remodeling Is Impaired in Patients with Alzheimer's Disease Carrying Apolipoprotein epsilon 4 Allele.
T. Arendt, C. Schindler, M. K. Bruckner, K. Eschrich, V. Bigl, D. Zedlick, and L. Marcova (1997)
J. Neurosci. 17, 516-529
   Abstract »    Full Text »    PDF »
Expression of Normal and Mutant Huntingtin in the Developing Brain.
P. G. Bhide, M. Day, E. Sapp, C. Schwarz, A. Sheth, J. Kim, A. B. Young, J. Penney, J. Golden, N. Aronin, et al. (1996)
J. Neurosci. 16, 5523-5535
   Abstract »    Full Text »    PDF »
Protein:Protein Interactions in Alzheimer's Disease and the CAG Triplet Repeat Diseases.
W.J. Strittmatter, J.R. Burke, V.S. DeSerrano, D.Y. Huang, W. Matthew, A.M. Saunders, B.L. Scott, J.M. Vance, K.H. Weisgraber, and A.D. Roses (1996)
Cold Spring Harb Symp Quant Biol 61, 597-605
   Abstract »    PDF »
Huntington's Disease.
J.F. Gusella, S. McNeil, F. Persichetti, J. Srinidhi, A. Novelletto, E. Bird, P. Faber, J.-P. Vonsattel, R.H. Myers, and M.E. MacDonald (1996)
Cold Spring Harb Symp Quant Biol 61, 615-626
   Abstract »    PDF »
Targeted Inactivation of the Mouse Huntington's Disease Gene Homolog Hdh.
M.E. MacDonald, M. Duyao, T. Calzonetti, A. Auerbach, A. Ryan, G. Barnes, J.K. White, W. Auerbach, J.-P. Vonsattel, J.F. Gusella, et al. (1996)
Cold Spring Harb Symp Quant Biol 61, 627-638
   Abstract »    PDF »
Correlation of Phosphorus-31 Magnetic Resonance Spectroscopy and Morphologic Findings in Alzheimer's Disease.
J. W. Pettegrew, K. Panchalingam, J. Moossy, J. Martinez, G. Rao, and F. Boller (1988)
Arch Neurol 45, 1093-1096
   Abstract »    PDF »
Cerebral Cation Shifts and Amino Acids in Huntington's Disease.
J. B. Gramsbergen, L. Veenma-Van der Duin, K. Venema, and J. Korf (1986)
Arch Neurol 43, 1276-1281
   Abstract »    PDF »
Molecular Genetics of Huntington's Disease.
J.F. Gusella, T.C. Gilliam, R.E. Tanzi, M.E. MacDonald, S.V. Cheng, M. Wallace, J. Haines, P.M. Conneally, and N.S. Wexler (1986)
Cold Spring Harb Symp Quant Biol 51, 359-364
   Abstract »    PDF »
Selective sparing of a class of striatal neurons in Huntington's disease.
R. Ferrante, N. Kowall, M. Beal, E. Richardson Jr, E. Bird, and J. Martin (1985)
Science 230, 561-563
   Abstract »    PDF »



To Advertise     Find Products


Science. ISSN 0036-8075 (print), 1095-9203 (online)