Characterization of mutant mice that express polyglutamine in cerebellar Purkinje cells

Brain Research

Volumn 1255, Issue , 2009, Pages 9-17

  Oue, Miho ^a   Mitsumura, Kazuhiro ^a   Torashima, Takashi ^a   Koyama, Chiho ^a   Yamaguchi, Haruyasu ^b   Furuya, Nobuhiko ^a   Hirai, Hirokazu ^a,c  

^a GUNMA UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b GUNMA UNIVERSITY   (Japan)

^c JAPAN SCIENCE AND TECHNOLOGY AGENCY   (Japan)

Author keywords

Cerebellum; Inclusion body; Polyglutamine; Purkinje cell; Spinocerebellar ataxia

Indexed keywords

CASPASE 3; POLYGLUTAMINE;

ANIMAL EXPERIMENT; APOPTOSIS; ARTICLE; CELL DEATH; CELL FUNCTION; CEREBELLUM; CONTROLLED STUDY; CYTOPLASM; ELECTRON MICROSCOPY; ELECTROPHYSIOLOGY; ELECTROSTIMULATION; IMMUNOHISTOCHEMISTRY; INNERVATION; MOUSE; MOUSE MUTANT; NERVE POTENTIAL; NERVE STIMULATION; NONHUMAN; PHENOTYPE; POSTSYNAPTIC POTENTIAL; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN EXPRESSION; PURKINJE CELL; TRANSGENIC MOUSE; UBIQUITINATION; WILD TYPE;

ANIMALS; CASPASE 3; CELL NUCLEUS; CEREBELLUM; ELECTROPHYSIOLOGY; EXCITATORY POSTSYNAPTIC POTENTIALS; IMMUNOHISTOCHEMISTRY; INCLUSION BODIES; MICE; MICE, INBRED STRAINS; MICE, TRANSGENIC; MICROSCOPY, ELECTRON; PEPTIDES; PURKINJE CELLS; UBIQUITINATION;

ANIMALIA; MUS; MUS MUSCULUS;

EID: 58949104590     PISSN: 00068993     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.brainres.2008.12.014     Document Type: Article

References (17)

1. Durr A., Stevanin G., Cancel G., Duyckaerts C., Abbas N., Didierjean O., Chneiweiss H., Benomar A., Lyon-Caen O., Julien J., Serdaru M., Penet C., Agid Y., and Brice A. Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features. Ann. Neurol. 39 (1996) 490-499
2. Fowler H.L. Machado-Joseph-Azorean disease. A ten-year study. Arch. Neurol. 41 (1984) 921-925
3. Hirai H., Miyazaki T., Kakegawa W., Matsuda S., Mishina M., Watanabe M., and Yuzaki M. Rescue of abnormal phenotypes of the delta2 glutamate receptor-null mice by mutant delta2 transgenes. EMBO Rep. 6 (2005) 90-95
4. Ito M. Historical review of the significance of the cerebellum and the role of Purkinje cells in motor learning. Ann. N. Y. Acad. Sci. 978 (2002) 273-288
5. Kawaguchi Y., Okamoto T., Taniwaki M., Aizawa M., Inoue M., Katayama S., Kawakami H., Nakamura S., Nishimura M., Akiguchi I., et al. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet. 8 (1994) 221-228
6. Orr H.T., and Zoghbi H.Y. Trinucleotide repeat disorders. Annu. Rev. Neurosci. 30 (2007) 575-621
7. Paulson H.L., Das S.S., Crino P.B., Perez M.K., Patel S.C., Gotsdiner D., Fischbeck K.H., and Pittman R.N. Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain. Ann. Neurol. 41 (1997) 453-462
8. Qin Q., Inatome R., Hotta A., Kojima M., Yamamura H., Hirai H., Yoshizawa T., Tanaka H., Fukami K., and Yanagi S. A novel GTPase, CRAG, mediates promyelocytic leukemia protein-associated nuclear body formation and degradation of expanded polyglutamine protein. J. Cell Biol. 172 (2006) 497-504
9. Sarna J.R., and Hawkes R. Patterned Purkinje cell death in the cerebellum. Prog. Neurobiol. 70 (2003) 473-507
10. Sato A., Shimohata T., Koide R., Takano H., Sato T., Oyake M., Igarashi S., Tanaka K., Inuzuka T., Nawa H., and Tsuji S. Adenovirus-mediated expression of mutant DRPLA proteins with expanded polyglutamine stretches in neuronally differentiated PC12 cells. Preferential intranuclear aggregate formation and apoptosis. Hum. Mol. Genet. 8 (1999) 997-1006
11. Schmidt T., Landwehrmeyer G.B., Schmitt I., Trottier Y., Auburger G., Laccone F., Klockgether T., Volpel M., Epplen J.T., Schols L., and Riess O. An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients. Brain Pathol. 8 (1998) 669-679
12. Sudarsky L., and Coutinho P. Machado-Joseph disease. Clin. Neurosci. 3 (1995) 17-22
13. Takahashi T., Kikuchi S., Katada S., Nagai Y., Nishizawa M., and Onodera O. Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum. Mol. Genet. 17 (2008) 345-356
14. Torashima T., Okoyama S., Nishizaki T., and Hirai H. In vivo transduction of murine cerebellar Purkinje cells by HIV-derived lentiviral vectors. Brain Res. 1082 (2006) 11-22
15. Torashima T., Koyama C., Iizuka A., Mitsumura K., Takayama K., Yanagi S., Oue M., Yamaguchi H., and Hirai H. Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia. EMBO Rep. 9 (2008) 393-399
16. Yamada M., Hayashi S., Tsuji S., and Takahashi H. Involvement of the cerebral cortex and autonomic ganglia in Machado-Joseph disease. Acta Neuropathol. 101 (2001) 140-144
17. Yoshizawa T., Yamagishi Y., Koseki N., Goto J., Yoshida H., Shibasaki F., Shoji S., and Kanazawa I. Cell cycle arrest enhances the in vitro cellular toxicity of the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. Hum. Mol. Genet. 9 (2000) 69-78