Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch

Journal of Cell Biology

Volumn 149, Issue 2, 2000, Pages 255-262

  Becker, Matthias ^a   Martin, Elke ^a   Schneikert, Jean ^a   Krug, Harald F ^a   Cato, Andrew C B ^a  

^a KARLSRUHE INSTITUTE OF TECHNOLOGY   (Germany)

Author keywords

Antiandrogens; Cytoplasmic inclusions; Ligand binding domain; Neurodegenerative disorder; Spinal and bulbar muscular atrophy

Indexed keywords

ANDROGEN RECEPTOR; POLYGLUTAMINE;

ANIMAL CELL; ARTICLE; CELL AGGREGATION; CELL COMPARTMENTALIZATION; CELL STRAIN COS1; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOPLASM; DEGENERATIVE DISEASE; GENE AMPLIFICATION; GENE MUTATION; KENNEDY DISEASE; LIGAND BINDING; NONHUMAN; PRIORITY JOURNAL; PROTEIN DOMAIN; X CHROMOSOME LINKED DISORDER;

ANIMALS; CELL NUCLEUS; COS CELLS; CYTOPLASM; GENE AMPLIFICATION; HUMANS; PEPTIDES; RECEPTORS, ANDROGEN; RECOMBINANT PROTEINS; SIGNAL TRANSDUCTION; TRANSFECTION;

ANIMALIA;

EID: 0034678342     PISSN: 00219525     EISSN: None     Source Type: Journal    
DOI: 10.1083/jcb.149.2.255     Document Type: Article

References (30)

1. Bingham, P.M., M.O. Scott, S. Wang, M.J. McPhaul, E.M. Wilson, J.Y. Garbern, D.E. Merry, and K.H. Fischbeck. 1995. Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice. Nat. Genet. 9:191-196.
2. Butler, R., P.N. Leigh, M.J. McPhaul, and J.-M. Gallo. 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.
3. Chamberlain, N.L., E.D. Driver, and R.L. Miesfeld. 1994. The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function. Nucleic Acids Res. 22:3181-3186.
4. Culig, Z., A. Hobisch, M.V. Cronauer, A.C.B. Cato, A. Hitmair, C. Radmayr, J. Eberle, G. Bartsch, and H. Klocker. 1993. Mutant androgen receptor detected in an advanced-stage prostatic carcinoma is activated by adrenal androgens and progesterone. Mol. Endocrinol 7:1541-1550.
5. Danek, A., T.N. Witt, K. Mann, H.U. Schweikert, G. Romalo, A.R. La Spada, and K.H. Fischbeck. 1994. Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy. Clin. Investig. 72:892-897.
6. 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.
7. DiFiglia, M., E. Sapp, K.O. Chase, S.W. Davies, G.P. Bates, J.P. Vonsattel, and N. Aronin. 1997. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuntes in brain. Science. 277:1990-1993.
8. Glenner, G.G. 1980. Amyloid deposits and amyloidosis. N. Engl. J. Med. 302: 1283-1292.
9. Gutekunst, C.-A., 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. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci. 19:2522-2534.
10. Huynh, D.P., M.R. Del Bigio, D.H. Ho, and S.M. Pulst. 1999. Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2. Ann. Neurol. 45:232-241.
11. Igarashi, S., R. Koide, T. Shimohata, M. Yamada, Y. Hayashi, H. Takano, H. Date, M. Oyake, T. Sato, A. Sato, et al. 1998. Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nat. Genet. 18:111-117.
12. Ishikawa, K., H. Fujigasaki, H. Saegusa, K. Ohwada, T. Fujita, H. Iwamoto, Y. Komatsuzaki, S. Toru, H. Toriyama, M. Watanabe, et al. 1999. Abundant expression and cytoplasmic aggregations of α1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6. Hum. Mol. Genet. 8:1185-1193.
13. Karpuj, M.V., H. Garren, H. Slunt, D.L. Price, J. Gusella, M.W. Becher, and L. Steinman. 1999. Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei. Proc. Natl. Acad. Sci. USA. 96:7388-7393.
14. Koo, E.H., P.T. Lansbury, Jr., and J.W. Kelly. 1999. Amyloid disease: abnormal protein aggregation in neurodegeneration. Proc. Natl. Acad. Sci. USA. 96: 9989-9990.
15. Kuil, C.W., and E. Mulder. 1994. Mechanism of antiandrogen action: conformational changes of the receptor. Mol. Cell. Endocrinol. 102:R1-R5.
16. Kuil, C.W., C.A. Berrevoets, and E. Mulder. 1995. Ligand-induced conformational alterations of the androgen receptor analyzed by limited trypsinization. J. Biol. Chem. 270:27569-27576.
17. Li, H., S.H. Li, A.L. Cheng, L. Mangiarini, G.P. Bates, and J. Li. 1999. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum. Mol. Genet. 8:1227-1236.
18. Li, M., Y. Nakagomi, Y. Kobayashi, D.E. Merry, F. Tanaka, M. Doyu, T. Mitsuma, Y. Hashizume, K.H. Fischbeck, and G. Sobue. 1998. Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy. Am. J. Pathol. 153:695-701.
19. Lin, X., C.J. Cummings, and H.Y. Zoghbi. 1999. Expanding our understanding of polyglutamine diseases through mouse models. Neuron. 24:499-502.
20. Merry, D.E., and K.H. Fischbeck. 1998. Genetics and molecular biology of the androgen receptor CAG repeat. In Genetic Instability and Hereditary Neurological Disease. R.D Wells and S.T. Warren, editors. Academic Press, New York. 101-111.
21. Neuschmid-Kaspar, F., A. Gast, H. Peterziel, J. Schneikert, A. Muigg. G. Ransmayr, H. Klocker, G. Bartsch, and A.C.B. Cato. 1996. CAG-repeat expansion in androgen receptor in Kennedy's disease is not a loss of function mutation. Mol. Cell. Endocrinol. 177:149-156.
22. Perez, M.K., H.L. Paulson, S.J. Pendse, S.J. Saionz, N.M. Bonini, and R.N. Pittman. 1998. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation, J. Cell Biol. 143:1457-1470.
23. Perutz, M.F. 1999. Glutamine repeats and neurodegenerative diseases: molecular aspects. Trends Biochem. Sci. 24:58-63.
24. Peterziel, H., S. Mink, A. Schonert, M. Becker, H. Klocker, and A.C.B. Cato. 1999. Rapid signalling by androgen receptor in prostate cancer cells. Oncogens. 18:6322-6329.
25. Ross, C.A. 1997. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? Neuron. 19:1147-1150.
26. 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.
27. Sisodia, S.S. 1998. Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial? Cell. 95:1-4.
28. Stenoien, D.L., C.J. Cummings, H.P. Adams, M.G. Mancini, K. Patel. G.N. DeMartino, M. Marcelli, N.L. Weigel, and M.A. Mancini. 1999. Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet. 8:731-741.
29. Trottier, Y., Y. Lutz, G. Stevanin, G. Imbert, D. Devys, G. Cancel, F. Saudou, C. Weber, G. David, L. Tora, et al. 1995. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature. 378:403-406.
30. Veldscholte, J., C.A. Berrevoets, A.O. Brinkmann, J.A. Grootegoed, and E. Mulder. 1992. Anti-androgens and the mutated androgen receptor of LNCaP cells: differential effects on binding affinity, heat-shock protein interaction, and transcription activation. Biochemistry. 31:2393-2399.