Kennedy's disease: Caspase cleavage of the androgen receptor is a crucial event in cytotoxicity

Journal of Neurochemistry

Volumn 72, Issue 1, 1999, Pages 185-195

  Ellerby, Lisa M ^a   Hackam, Abigail S ^d   Propp, Stephanie S ^a   Ellerby, H Michael ^a   Rabizadeh, Shahrooz ^a,b   Cashman, Neil R ^f   Trifiro, Mark A ^e   Pinsky, Leonard ^e   Wellington, Cheryl L ^d   Salvesen, Guy S ^a   Hayden, Michael R ^d   Bredesen, Dale E ^a,c  

^a BURNHAM INSTITUTE   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

^d UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^e MCGILL UNIVERSITY   (Canada)

^f UNIVERSITY OF TORONTO   (Canada)

Author keywords

Aggregates; Androgen receptor; Caspase; Kennedy's disease; Triplet repeat disease

Indexed keywords

ANDROGEN RECEPTOR; PROTEINASE;

ARTICLE; CYTOTOXICITY; ENZYME ACTIVITY; HUMAN; HUMAN CELL; KENNEDY DISEASE; NERVE DEGENERATION; PATHOPHYSIOLOGY; PRIORITY JOURNAL;

CARCINOGENS; CASPASES; CATALYTIC DOMAIN; CELL DEATH; CELL NUCLEUS; CELLS, CULTURED; CYTOTOXINS; ENZYME ACTIVATION; FETUS; GENE EXPRESSION; KIDNEY; MUSCULAR ATROPHY, SPINAL; MUTAGENESIS; NEURONS; PEPTIDES; RECEPTORS, ANDROGEN; TESTOSTERONE; TRANSFECTION; TRINUCLEOTIDE REPEATS;

EID: 0032898311     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1471-4159.1999.0720185.x     Document Type: Article

References (48)

1. Abdullah A. A. R., Trifiro M. A., Panet-Raymond V., Alvarado C., de Tourreil S., Frankel D., Schipper H. M., and Pinsky L. (1998) Spinobulbar muscular atrophy: polyglutamine-expanded androgen receptor is proteolytically resistant in vitro and processed abnormally in transfected cells. Hum. Mol. Genet. 7, 379-384.
2. Brooks B. P., Paulsen H. L., Merry D. E., Salazar-Grueso E. F., Brinkmann A. O., Wilson E. M., and Fishbeck K. H. (1997) Characterization of an expanded glutamine repeat androgen receptor in a neuronal cell culture system. Neurbiol. Dis. 4, 313-323.
3. Butler R., Leigh P. N., McPhaul M. J., and Gallo J.-M. (1998) Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy. Hum. Mol. Genet. 7, 121-127.
4. Cardone M. H., Salvesen G. S., Widmann C., Johnson G., and Frisch S. M. (1997) The regulation of anoikis: MEKK-1 activation requires cleavage by caspases. Cell 90, 315-323.
5. Cashman N. R., Durham H. D., Blusztajn J. K., Oda K., Tabira T., Shaw I. T., Dahrouge S., and Antel J. P. (1992) Neuroblastoma × spinal cord (NSC) hybrid cell lines resemble developing motor neurons. Dev. Dyn. 194, 209-221.
6. David G., Abbas N., Stevanin G., Durr A., Yvert G., Cancel G., Weber C., Imbert G., Saudou F., et al. (1997) Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion. Nat. Genet. 17, 65-70.
7. Davies S. W., Turmaine M., Cozens B. A., DiFiglia M., Sharp A. H., Ross C. R., Scherzinger E., Wanker E. E., Mangiarini L., and Bates G. P. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537-548.
8. Deveraux Q. L., Takahashi R., Salvesen G. S., and Reed J. C. (1997) X-linked IAP is direct inhibitor of cell death proteases. Nature 388, 300-304.
9. DiFiglia M., Sapp E., Chase K. O., Davies S. W., Bates G. P., Vonsattel J. P., and Aronin N. (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuntes in brain. Science 277, 1990-1993.
10. Ellerby H. M., Martin S. J., Ellerby L. M., Naiem S. S., Rabizadeh S., Salvesen G. S., Casiano N. R., Green D. R., and Bredesen D. E. (1997) Establishment of a cell-free system of apoptosis: comparison of premitochondrial, mitochondrial, and post mitochondrial phases. J. Neurosci. 17, 6165-6178.
11. Fischbeck K. H. (1997) Kennedy disease. J. Inherit. Metab. Dis. 20, 152-158.
12. Goldberg Y. P., Nicholson D. W., Rasper D. M., Kalchman M. A., Koide H. B., Graham R. K., Bromm M., Kazemi-Esfarjani P., Thornberry N. A., Vaillancourt J. P., and Hayden M. R. (1996) Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat. Genet. 13, 442-449.
13. Howard A. D., Kostura M. J., Thornberry N., Ding G. J., Limjuco G., Weidner J., Salley J. P., Hogquist K. A., Chaplin D. D., Mumford R. A., et al. (1991) IL-1-converting enzyme requires aspartic acid residues for processing of the IL-1 beta precursor at two distinct sites and does not cleave 31-kDa IL-1 alpha. J. Immunol. 147, 2964-2969.
14. Huntington's Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72, 971-983.
15. Igarashi S., Koide R., Shimohata T., Yamada M., Hayashi Y., Takano H., Date H., Oyake M., Sato T., Sato A., Egawa S., Ikeuchi T., Tanaka H., Nakano R., Tanaka K., Hozumi I., Inuzuka T., Takahashi H., and Tsuji S. (1998) Suppression of aggregate and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nat. Genet. 18, 111-117.
16. Ikeda H., Yamaguchi M., Sugai S., Aze Y., Narumiya S., and Kakizuka A. (1996) Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nat. Genet. 13, 196-202.
17. Imbert G., Saudou F., Yvert G., Devys D., Trottier Y., Garnier J. M., Weber C., Mandel J. L., Cancel G., Abbas N., Durr A., Didierjean O., Stevanin G., Agid Y., and Brice A. (1996) Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nat. Genet. 14, 285-291.
18. Jordan M., Schallhorn A., and Wurm F. M. (1996) Transfecting mammalian cells: optimization of critical parameters affecting calciumphosphate precipitate formation. Nucleic Acids Res. 24, 596-601.
19. Kawaguchi Y., Okamoto T., Taniwaki M., Aizawa M., Inoue M., Katayama S., Kawakami H., Nakamura S., Nishimura M., Akiguchi I., Kimura J., Narumiya S., and Kakizuka A. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet. 8, 221-227.
20. Kayalar C., Ord T., Testa M. P., Zhong L. T., and Bredesen D. E. (1996) Cleavage of actin by interleukin 1 beta-converting enzyme to reverse DNase I inhibition. Proc. Natl. Acad. Sci. USA 93, 2234-2238.
21. n-expanded neuronopathies. Hum. Mol. Genet. 4, 523-527.
22. Kemppainen J. A., Lane M. V., Sar M., and Wilson E. M. (1992) Androgen receptor phosphorylation, turnover, nuclear transport, and transcriptional activation. J. Biol. Chem. 267, 968-974.
23. Kerr J. F. R., Wyllie A. H., and Currie A. R. (1972) Apoptosis: a basic biological phenomenon with wide-ranging implications in tissue kinetics. Br. J. Cancer 26, 239-257.
24. Kim T.-W., Pettingell W. H., Jung Y.-K., Kovacs D. M., and Tanzi R. E. (1997) Alternative cleavage of Alzheimer-associated presenilins during apoptosis by a caspase-3 family protease. Science 277, 373-376.
25. Koide R., Ikeuchi T., Onodera O., Tanaka H., Igarashi S., Endo K., Takahashi H., Kondo R., Ishikawa A., Hayashi T., et al. (1994) Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nat. Genet. 6, 9-13.
26. La Spada A. R., Wilson E. M., Lubahn D. B., Harding A. E., and Fischbeck K. H. (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352, 77-79.
27. Lockshin R. A. and Williams C. M. (1964) Programmed cell death. II. Endocrine potentiation of the breakdown of the intersegmental muscles of silkmoths. J. Insect Physiol. 10, 643-649.
28. Martindale D., Hackam A., Wieczorek A., Ellerby L. M., Wellington C., McCutcheon K., Devon R., Sinaraja R., Bredesen D., Tufaro F., and Hayden M. R. (1998) Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat. Genet. 18, 150-154.
29. McGahon A. J., Martin S. J., Bissonnette R. P., Mahboubi R. P., Mahboubi A., Shi Y., Mogil R. J., Nishioka W. K., and Green D. R. (1995) The end of the (cell) line: methods for the study of apoptosis in vitro. Methods Cell Biol. 46, 153-185.
30. Miyashita T., Okamuraoho Y., Mito Y., Nagafuchi S., and Yamada M. (1997) Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis. J. Biol. Chem. 272, 29238-29242.
31. Nance M. A. (1997) Clinical aspects of CAG repeat diseases. Brain Pathol. 7, 881-900.
32. Orr H. T., Chung M., Banfi S., Kwiatkowski T. J., Servadio A., Beaudet A. L., McCali A. E., Duvick L. A., Ranum L. P. W., and Zoghbi H. Y. (1993) Expansion of the unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat. Genet. 4, 221-226.
33. Orth K., O'Rourke K., Salvesen G. S., and Dixit V. M. (1996) Molecular ordering of apoptotic mammalian CED-3/ICE-like proteases. J. Biol. Chem. 274, 20977-20980.
34. Paulson H. L., Perez M. K., Trottier Y., Trojanowski S. H., Subramony S. H., Das S. S., Vig P., Mandel J.-L., Fischbeck K. H., and Pittman R. N. (1997) Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333-334.
35. Perutz M. F. (1996) Glutamine repeats and inherited neurodegenerative diseases: molecular aspects. Curr. Opin. Struct. Biol. 6, 848-858.
36. Ross C. A. (1995) When more is less: pathogenesis of glutamine repeat neurodegenerative diseases. Neuron 15, 493-496.
37. Roy N., Mahadevan M. S., McLean M., Shutler G., Yaraghi Z., Farhani R., Baird S., Besner-Johnston A., Lefebvre C., Kang X., et al. (1995) The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy. Cell 80, 167-178.
38. Salvesen G. S. and Dixit V. M. (1997) Caspases: intracellular signaling by proteolysis. Cell 91, 443-446.
39. Salvesen G. and Enghild J. (1990) An unusual specificity in the activation of neutrophil serine protease zymogens. Biochemistry 29, 5304-5308.
40. Sanpei K., Takano H., Igarashi S., Sato T., Oyake M., Sasaki H., Wakisaka A., Tashiro K., Ishida Y., Ikeuchi T., Koide R., Saito M., Sato A., and Tanaka T. (1996) Identification of the gene for spinocerebellar ataxia type 2 (SCA2) using a direct identification of repeat expansion and cloning technique (DIRECT). Nat. Genet. 14, 277-284.
41. Scherzinger E., Lurz R., Turmaine M., Mangiarini L., Hollenbach B., Hasenbank R., Bates G. P., Davies S. W., Lehrach H., and Wanker E. E. (1997) Huntington-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549-558.
42. Wellington C. L., Ellerby L. M., Hackam A. S., Margolis R. L., Trifiro M. A., Singaraja R., McCutcheon K., Salvesen G. S., Propp S. S., Bromm M., Rowland K. J., Zhang T. Q., Rasper D., Roy S., Thornberry N., Pinsky L., Kakizuka A., Ross C. A., Nicholson D. W., Bredesen D. E., and Hayden M. R. (1998) Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J. Biol. Chem. 273, 9158-9167.
43. Wolozin B., Iwasaki K., Vito P., Ganjei J. K., Lacana E., Sunderland T., Zhao B., Kusiak J. W., Wasco W., and D'Adamio L. (1996) Participation of presenilin 2 in apoptosis: enhanced basal activity conferred by an Alzheimer mutation. Science 274, 1710-1713.
44. Yamatsuji T., Matsui T., Okamoto T., Komatsuzaki K., Takeda S., Fukumoto H., Iwatsubo T., Suzuki N., Asami-Odaka A., and Ireland S. (1996) G protein-mediated neuronal DNA fragmentation induced by familial Alzheimer's disease-associated mutants of APP. Science 272, 1349-1352.
45. Zhivotovsky B., Burgess D. H., and Orrenius S. (1996) Proteases in apoptosis. Experientia 52, 968-978.
46. Zhivotovsky B., Gahm A., and Orrenius S. (1997) Two different proteases are involved in the proteolysis of Iambi during apoptosis. Biochem. Biophys. Res. Commun. 233, 96-101.
47. Zhou Q., Snipas S., Orth K., Dixit V. M., and Salvesen G. S. (1997) Target protease specificity of the viral serpin CrmA. Analysis of five caspases. J. Biol. Chem. 272, 7797-7800.
48. 1A-voltage-dependent calcium channel. Nat. Genet. 15, 62-69.