Attenuated nuclear shrinkage in neurons with nuclear aggregates - A morphometric study on pontine neurons of Machado-Joseph disease brains

Experimental Neurology

Volumn 178, Issue 1, 2002, Pages 124-128

  Uchihara, Toshiki ^a   Iwabuchi, Kiyoshi ^b   Funata, Nobuaki ^c   Yagishita, Saburo ^b  

^a TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

^b KANAGAWA REHABILITATION CENTER   (Japan)

^c TOKYO METROPOLITAN KOMAGOME HOSPITAL   (Japan)

Author keywords

Intranuclear inclusions; Morphometry; Neurodegeneration; Nuclear size; Ubiquitin

Indexed keywords

ARTICLE; ATTENUATION; AUTOPSY; BRAIN TISSUE; CASE REPORT; CELL NUCLEUS; CELL SHAPE; CELL SIZE; CONTROLLED STUDY; FEMALE; HUMAN; HUMAN TISSUE; MACHADO JOSEPH DISEASE; MALE; MORPHOLOGY; MORPHOMETRICS; NERVE CELL; PONS; PRIORITY JOURNAL; TISSUE SECTION;

EID: 0036445864     PISSN: 00144886     EISSN: None     Source Type: Journal    
DOI: 10.1006/exnr.2002.8028     Document Type: Article

References (23)

1. Bots, G. T. A. M., and G. W. Bruyn. 1981. Neuropathological changes of the nucleus accumbens in Huntington's chorea. Acta Neuropathol. 55: 21-22.
2. Cummings, C. J., M. A. Mancini, B. Antalffy, D. B. DeFranco, H. T. Orr, and H. Y. Zoghbi. 1998. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19: 148-154.
3. Cummings, C. J., E. Reinstein, Y. Sun, B. Antalffy, Y.-H. Jiang, A. Ciechanover, H. T. Orr, A. L. Beaudet, and H. Y. Zoghbi. 1999. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron 24: 879-892.
4. Davies, S. W., M. Turmaine, B. A. Cozens, M. DiFiglia, A. H. Sharp, C. A. Ross, E. Scherzinger, E. E. Wanker, L. Mangiarini, and G. P. Bates. 1997. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90: 537-548.
5. Fujigasaki, H., T. Uchihara, S. Koyano, K. Iwabuchi, S. Yagishita, T. Makifuchi, A. Nakamura, K. Ishida, S. Toru, S. Hirai, K. Ishikawa, T. Tanabe, and H. Mizusawa. 2000. Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains. Exp. Neurol. 165: 248-256.
6. Hertwig, R. 1903. Ueber Korrelation von Zell- und Kerngrösse und ihre Bedeutung für die deschlechtliche Differenzierung und die Teilung der Zelle. Biol. Zbl. 23: 49-119.
7. Iwabuchi, K., K. Tsuchiya, T. Uchihara, and S. Yagishita. 1999. Autosomal dominant spinocerebellar degenerations. Clinical, pathological and genetic correlations. Rev. Neurol. (Paris) 155: 255-270.
8. Kim, M., H. S. Lee, G. LaForet, C. McIntyre, E. J. Matin, P. Chang, T. W. Kim, M. Williams, P. H. Reddy, D. Tagle, F. M. Boyce, L. Won, A. Heller, N. Aronin, and M. DiFiglia. 1999. Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition. J. Neurochem. 19: 964-973.
9. Klement, I. A., P. J. Skinner, M. D. Kaytor, H. Yi, S. M. Hersch, H. B. Clark, H. Y. Zoghbi, and H. T. Orr. 1998. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95: 41-53.
10. Koyano, S., K. Iwabuchi, S. Yagishita, Y. Kuroiwa, and T. Uchihara. 2002. Paradoxical absence of nuclear inclusion in cerebellar Purkinje cells of hereditary ataxias linked to CAG expansion. J. Neurol. Neurosurg. Psychiatry 73: 453-455.
11. Kuemmerle, S., C.-A. Gutekunst, A. M. Klein, X.-J. Li, S.-H. Li, M. F. Beal, S. M. Hersche, and R. J. Ferrante. 1999. Huntingtin aggregates may not predict neuronal death in Huntington's disease. Ann. Neurol. 46: 842-849.
12. Montagne, J. 2000. Genetic and molecular mechanisms of cell size control. Mol. Cell. Biol. Res. Commun. 4: 195-202.
13. Ona, V. O., M. Li, J. P. Vonsattel, L. J. Andrews, S. Q. Khan, W. M. Chung, A. S. Frey, A. S. Menon, X.-J. Li, P. E. Stieg, J. Yuan, J. B. Penney, A. B. Young, J.-H. J.Cha, and R. M. Friedlander. 1999. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 399: 263-267.
14. Ordway, J. M., S. Tallaksen-Greene, C.-A. Gutekunst, E. M. Bernstein, J. A. Cearley, H. W. Wiener, L. S. Dure IV, R. Lindsey, S. M. Hersch, R. S. Jope, R. L. Albin, and P. J. Detloff. 1997. Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 91: 753-763.
15. Roos, R. A. C., and G. T. A. M. Bots. 1983. Nuclear membrane indentations in Huntington's chorea. J. Neurol. Sci. 61: 37-47.
16. Saudou, F., S. Finkbeiner, D. Devys, and M. E. Greenberg. 1998. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95: 55-66.
17. Sisodia, S. S. 1998. Nuclear inclusions in glutamine repeat disorders: Are they pernicious, coincidental, or beneficial? Cell 95: 1-4.
18. Takahashi, H., S. Egawa, Y.-S. Piao, S. Hayashi, M. Yamada, K. Oyanagi, and S. Tsuji. 2001. Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: Ultrastructural and morphometric studies of cerebellar granule cells. Brain Res. 919: 12-19.
19. Takahashi, J., T, Fukuda, J. Tanaka, H. Fujigasaki, M. Minamitani, and T. Uchihara. 2000. Neuronal intranuclear hyaline inclusion disease with polyglutamine immunoreactive inclusions. Acta Neuropathol. 99: 589-594.
20. Takahashi, J., J. Tanaka, K. Arai, N. Funata, T. Hattori, T. Fukuda, H. Fujigasaki, and T. Uchihara. 2001. Recruitment of non-expanded polyglutamine proteins in intranuclear aggre-ages of neuronal intranuclear hyaline inclusion disease. J. Neu-ropathol. Exp. Neurol. 60: 369-376.
21. Watase, K., E. J. Weeber, B. Xu, B. Antalffy, L. Yuva-Paylor, K. Hashimoto, M. Kano, R. Atkinson, Y. Sun, D. L. Armstrong, J. D. Sweatt, H. T. Orr, R. Paylor, and H. Y. Zoghbi. 2002. A long CAG repeat in the mouse sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration. Neuron 34: 905-919.
22. Yamamoto, A., J. J. Lucas, R. and Hen. 2000. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101: 57-66.
23. Zoghbi, H. Y., and H. T. Orr. 2000. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci. 2000: 217-47.