Histone acetylation, acetyltransferases, and ataxia - Alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disorders

Advances in Protein Chemistry and Structural Biology

Volumn 79, Issue C, 2010, Pages 165-203

  McCullough, Shaun D ^a   Grant, Patrick A ^a  

^a UNIVERSITY OF VIRGINIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 78650101277     PISSN: 18761623     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S1876-1623(10)79005-2     Document Type: Chapter

References (104)

1. R. Balasubramanian , M.G. Pray-Grant , W. Selleck , P.A. Grant , and S. Tan Role of the Ada2 and Ada3 transcriptional coactivators in histone acetylation J. Biol. Chem. 277 2002 7989 7995
2. K. Balasubramanyam , R.A. Varier , M. Altaf , V. Swaminathan , N.B. Siddappa , and U. Ranga Curcumin, a novel p300/CREB-binding protein-specific inhibitor of acetyltransferase, represses the acetylation of histone/nonhistone proteins and histone acetyltransferase-dependent chromatin transcription J. Biol. Chem. 279 2004 51163 51171
3. N.A. Barlev , A.V. Emelyanov , P. Castagnino , P. Zegerman , A.J. Bannister , and M.A. Sepulveda A novel human Ada2 homologue functions with Gcn5 or Brg1 to coactivate transcription Mol. Cell. Biol. 23 2003 6944 6957
4. A. Benomar , L. Krols , G. Stevanin , G. Cancel , E. LeGuern , and G. David The gene for autosomal dominant cerebellar ataxia with pigmentary macular dystrophy maps to chromosome 3p12-p21.1 Nat. Genet. 10 1995 84 88
5. A. Benomar , E. Le Guern , A. Durr , H. Ouhabi , G. Stevanin , and M. Yahyaoui Autosomal-dominant cerebellar ataxia with retinal degeneration (ADCA type II) is genetically different from ADCA type I Ann. Neurol. 35 1994 439 444
6. S.J. Berke , Y. Chai , G.L. Marrs , H. Wen , and H.L. Paulson Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3 J. Biol. Chem. 280 2005 32026 32034
7. J.E. Brownell , and C.D. Allis An activity gel assay detects a single, catalytically active histone acetyltransferase subunit in Tetrahymena macronuclei Proc. Natl. Acad. Sci. U.S.A. 92 1995 6364 6368
8. J.E. Brownell , J. Zhou , T. Ranalli , R. Kobayashi , D.G. Edmondson , and S.Y. Roth Tetrahymena histone acetyltransferase A: a homolog to yeast Gcn5p linking histone acetylation to gene activation Cell 84 1996 843 851
9. B.G. Burnett , and R.N. Pittman The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation Proc. Natl. Acad. Sci. U.S.A. 102 2005 4330 4335
10. R. Candau , P.A. Moore , L. Wang , N. Barlev , C.Y. Ying , and C.A. Rosen Identification of human proteins functionally conserved with the yeast putative adaptors ADA2 and GCN5 Mol. Cell. Biol. 16 1996 593 602
11. S. Chen , G.H. Peng , X. Wang , A.C. Smith , S.K. Grote , and B.L. Sopher Interference of Crx-dependent transcription by ataxin-7 involves interaction between the glutamine regions and requires the ataxin-7 carboxy-terminal region for nuclear localization Hum. Mol. Genet. 13 2004 53 67
12. A. Ciurciu , O. Komonyi , T. Pankotai , and I.M. Boros The Drosophila histone acetyltransferase Gcn5 and transcriptional adaptor Ada2a are involved in nucleosomal histone H4 acetylation Mol. Cell. Biol. 26 2006 9413 9423
13. A.J. Courey , and R. Tjian Analysis of Sp1 in vivo reveals multiple transcriptional domains, including a novel glutamine-rich activation motif Cell 55 1988 887 898
14. S.K. Custer , G.A. Garden , N. Gill , U. Rueb , R.T. Libby , and C. Schultz Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport Nat. Neurosci. 9 2006 1302 1311
15. J.A. Daniel , M.S. Torok , Z.W. Sun , D. Schieltz , C.D. Allis , and J.R. Yates 3rd Deubiquitination of histone H2B by a yeast acetyltransferase complex regulates transcription J. Biol. Chem. 279 2004 1867 1871
16. G. David , N. Abbas , G. Stevanin , A. Durr , G. Yvert , and G. Cancel Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion Nat.Genet. 17 1997 65 70
17. G. David , P. Giunti , N. Abbas , P. Coullin , G. Stevanin , and W. Horta The gene for autosomal dominant cerebellar ataxia type II is located in a 5-cM region in 3p12-p13: genetic and physical mapping of the SCA7 locus Am. J. Hum. Genet. 59 1996 1328 1336
18. C. Dhalluin , J.E. Carlson , L. Zeng , C. He , A.K. Aggarwal , and M.M. Zhou Structure and ligand of a histone acetyltransferase bromodomain Nature 399 1999 491 496
19. A. Eberharter , D.E. Sterner , D. Schieltz , A. Hassan , J.R. Yates 3rd , and S.L. Berger The ADA complex is a distinct histone acetyltransferase complex in Saccharomyces cerevisiae Mol. Cell. Biol. 19 1999 6621 6631
20. L.M. Ellerby , A.S. Hackam , S.S. Propp , H.M. Ellerby , S. Rabizadeh , and N.R. Cashman Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity J. Neurochem. 72 1999 185 195
21. B.O. Evert , J. Araujo , A.M. Vieira-Saecker , R.A. de Vos , S. Harendza , and T. Klockgether Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation J. Neurosci. 26 2006 11474 11486
22. A.M. Gamper , J. Kim , and R.G. Roeder The STAGA subunit ADA2b is an important regulator of human GCN5 catalysis Mol. Cell. Biol. 29 2009 266 280
23. G.A. Garden , R.T. Libby , Y.H. Fu , Y. Kinoshita , J. Huang , and D.E. Possin Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice J. Neurosci. 22 2002 4897 4905
24. T. Georgakopoulos , and G. Thireos Two distinct yeast transcriptional activators require the function of the GCN5 protein to promote normal levels of transcription EMBO J. 11 1992 4145 4152
25. H.P. Gerber , K. Seipel , O. Georgiev , M. Hofferer , M. Hug , and S. Rusconi Transcriptional activation modulated by homopolymeric glutamine and proline stretches Science 263 1994 808 811
26. P.A. Grant , and S.L. Berger Histone acetyltransferase complexes Semin. Cell Dev. Biol. 10 1999 169 177
27. P.A. Grant , L. Duggan , J. Cote , S.M. Roberts , J.E. Brownell , and R. Candau Yeast Gcn5 functions in two multisubunit complexes to acetylate nucleosomal histones: characterization of an Ada complex and the SAGA (Spt/Ada) complex Genes Dev. 11 1997 1640 1650
28. P.A. Grant , A. Eberharter , S. John , R.G. Cook , B.M. Turner , and J.L. Workman Expanded lysine acetylation specificity of Gcn5 in native complexes J. Biol. Chem. 274 1999 5895 5900
29. P.A. Grant , D. Schieltz , M.G. Pray-Grant , D.J. Steger , J.C. Reese , and J.R. Yates 3rd A subset of TAF(II)s are integral components of the SAGA complex required for nucleosome acetylation and transcriptional stimulation Cell 94 1998 45 53
30. S. Guelman , T. Suganuma , L. Florens , S.K. Swanson , C.L. Kiesecker , and T. Kusch Host cell factor and an uncharacterized SANT domain protein are stable components of ATAC, a novel dAda2A/dGcn5-containing histone acetyltransferase complex in Drosophila Mol. Cell. Biol. 26 2006 871 882
31. D. Helmlinger , G. Abou-Sleymane , G. Yvert , S. Rousseau , C. Weber , and Y. Trottier Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model J. Neurosci. 24 2004 1881 1887
32. D. Helmlinger , S. Hardy , G. Abou-Sleymane , A. Eberlin , A.B. Bowman , and A. Gansmuller Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction PLoS Biol. 4 2006 e67
33. D. Helmlinger , S. Hardy , S. Sasorith , F. Klein , F. Robert , and C. Weber Ataxin-7 is a subunit of GCN5 histone acetyltransferase-containing complexes Hum. Mol. Genet. 13 2004 1257 1265
34. A.K. Hennig , G.H. Peng , and S. Chen Regulation of photoreceptor gene expression by Crx-associated transcription factor network Brain Res. 1192 2008 114 133
35. K.W. Henry , A. Wyce , W.S. Lo , L.J. Duggan , N.C. Emre , and C.F. Kao Transcriptional activation via sequential histone H2B ubiquitylation and deubiquitylation, mediated by SAGA-associated Ubp8 Genes Dev. 17 2003 2648 2663
36. J. Horiuchi , N. Silverman , G.A. Marcus , and L. Guarente ADA3, a putative transcriptional adaptor, consists of two separable domains and interacts with ADA2 and GCN5 in a trimeric complex Mol. Cell. Biol. 15 1995 1203 1209
37. T. Ito , T. Chiba , R. Ozawa , M. Yoshida , M. Hattori , and Y. Sakaki A comprehensive two-hybrid analysis to explore the yeast protein interactome Proc. Natl. Acad. Sci. U.S.A. 98 2001 4569 4574
38. R.H. Jacobson , A.G. Ladurner , D.S. King , and R. Tjian Structure and function of a human TAFII250 double bromodomain module Science 288 2000 1422 1425
39. N. James , E. Landrieux , and M.A. Collart A SAGA-independent function of SPT3 mediates transcriptional deregulation in a mutant of the Ccr4-not complex in Saccharomyces cerevisiae Genetics 177 2007 123 135
40. H. Kikuchi , Y. Takami , and T. Nakayama GCN5: a supervisor in all-inclusive control of vertebrate cell cycle progression through transcription regulation of various cell cycle-related genes Gene 347 2005 83 97
41. A. Kohler , P. Pascual-Garcia , A. Llopis , M. Zapater , F. Posas , and E. Hurt The mRNA export factor Sus1 is involved in Spt/Ada/Gcn5 acetyltransferase-mediated H2B deubiquitinylation through its interaction with Ubp8 and Sgf11 Mol. Biol. Cell 17 2006 4228 4236
42. A. Kohler , M. Schneider , G.G. Cabal , U. Nehrbass , and E. Hurt Yeast Ataxin-7 links histone deubiquitination with gene gating and mRNA export Nat. Cell. Biol. 10 2008 707 715
43. M.H. Kuo , J.E. Brownell , R.E. Sobel , T.A. Ranalli , R.G. Cook , and D.G. Edmondson Transcription-linked acetylation by Gcn5p of histones H3 and H4 at specific lysines Nature 383 1996 269 272
44. T. Kusch , S. Guelman , S.M. Abmayr , and J.L. Workman Two Drosophila Ada2 homologues function in different multiprotein complexes Mol. Cell. Biol. 23 2003 3305 3319
45. A.R. La Spada , Y.H. Fu , B.L. Sopher , R.T. Libby , X. Wang , and L.Y. Li Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7 Neuron 31 2001 913 927 (Pubitemid 32925279)
46. K.K. Lee , S.K. Swanson , L. Florens , M.P. Washburn , and J.L. Workman Yeast Sgf73/Ataxin-7 serves to anchor the deubiquitination module into both SAGA and Slik(SALSA) HAT complexes Epigenetics Chromatin. 2 2009 2
47. C. Leranth , and J. Hamori "Dark" Purkinje cells of the cerebellar cortex Acta Biol. Acad. Sci. Hung. 21 1970 405 419
48. F. Li , T. Macfarlan , R.N. Pittman , and D. Chakravarti Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities J. Biol. Chem. 277 2002 45004 45012
49. K. Lindblad , M.L. Savontaus , G. Stevanin , M. Holmberg , K. Digre , and C. Zander An expanded CAG repeat sequence in spinocerebellar ataxia type 7 Genome Res. 6 1996 965 971
50. K. Luger , A.W. Mader , R.K. Richmond , D.F. Sargent , and T.J. Richmond Crystal structure of the nucleosome core particle at 2.8 A resolution Nature 389 1997 251 260
51. M.G. Marcu , Y.J. Jung , S. Lee , E.J. Chung , M.J. Lee , and J. Trepel Curcumin is an inhibitor of p300 histone acetylatransferase Med. Chem. 2 2006 169 174
52. G.A. Marcus , N. Silverman , S.L. Berger , J. Horiuchi , and L. Guarente Functional similarity and physical association between GCN5 and ADA2: putative transcriptional adaptors EMBO J. 13 1994 4807 4815
53. E. Martin-Aparicio , A. Yamamoto , F. Hernandez , R. Hen , J. Avila , and J.J. Lucas Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease J. Neurosci. 21 2001 8772 8781
54. E. Martinez , T.K. Kundu , J. Fu , and R.G. Roeder A human SPT3-TAFII31-GCN5-L acetylase complex distinct from transcription factor IID J. Biol. Chem. 273 1998 23781 23785
55. E. Martinez , V.B. Palhan , A. Tjernberg , E.S. Lymar , A.M. Gamper , and T.K. Kundu Human STAGA complex is a chromatin-acetylating transcription coactivator that interacts with pre-mRNA splicing and DNA damage-binding factors in vivo Mol. Cell. Biol. 21 2001 6782 6795
56. T. Maurice , F. Duclot , J. Meunier , G. Naert , L. Givalois , and J. Meffre Altered memory capacities and response to stress in p300/CBP-associated factor (PCAF) histone acetylase knockout mice Neuropsychopharmacology 33 2008 1584 1602
57. B.M. Mayr , E. Guzman , and M. Montminy Glutamine rich and basic region/leucine zipper (bZIP) domains stabilize cAMP-response element-binding protein (CREB) binding to chromatin J. Biol. Chem. 280 2005 15103 15110
58. A. McCampbell , A.A. Taye , L. Whitty , E. Penney , J.S. Steffan , and K.H. Fischbeck Histone deacetylase inhibitors reduce polyglutamine toxicity Proc. Natl. Acad. Sci. U.S.A. 98 2001 15179 15184
59. S.J. McMahon , M.G. Pray-Grant , D. Schieltz , J.R. Yates 3rd , and P.A. Grant Polyglutamine-expanded spinocerebellar ataxia-7 protein disrupts normal SAGA and SLIK histone acetyltransferase activity Proc. Natl. Acad. Sci. U.S.A. 102 2005 8478 8482
60. M. Minamiyama , M. Katsuno , H. Adachi , M. Waza , C. Sang , and Y. Kobayashi Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy Hum. Mol. Genet. 13 2004 1183 1192
61. P.J. Mitchell , and R. Tjian Transcriptional regulation in mammalian cells by sequence-specific DNA binding proteins Science 245 1989 371 378
62. S. Muratoglu , S. Georgieva , G. Papai , E. Scheer , I. Enunlu , and O. Komonyi Two different Drosophila ADA2 homologues are present in distinct GCN5 histone acetyltransferase-containing complexes Mol. Cell. Biol. 23 2003 306 321
63. Y. Nagai , and H.A. Popiel Conformational changes and aggregation of expanded polyglutamine proteins as therapeutic targets of the polyglutamine diseases: exposed beta-sheet hypothesis Curr. Pharm. Des. 14 2008 3267 3279
64. Z. Nagy , and L. Tora Distinct GCN5/PCAF-containing complexes function as co-activators and are involved in transcription factor and global histone acetylation Oncogene 26 2007 5341 5357
65. F.C. Nucifora Jr , M. Sasaki , M.F. Peters , H. Huang , J.K. Cooper , and M. Yamada Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity Science 291 2001 2423 2428
66. V.V. Ogryzko , T. Kotani , X. Zhang , R.L. Schiltz , T. Howard , and X.J. Yang Histone-like TAFs within the PCAF histone acetylase complex Cell 94 1998 35 44 (Pubitemid 28347467)
67. H.T. Orr , and H.Y. Zoghbi Trinucleotide repeat disorders Annu. Rev. Neurosci. 30 2007 575 621
68. M.A. Osley Regulation of histone H2A and H2B ubiquitylation Brief Funct. Genomic Proteomic. 5 2006 179 189
69. V.B. Palhan , S. Chen , G.H. Peng , A. Tjernberg , A.M. Gamper , and Y. Fan Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration Proc. Natl. Acad. Sci. U.S.A. 102 2005 8472 8477
70. M.G. Pray-Grant , D. Schieltz , S.J. McMahon , J.M. Wood , E.L. Kennedy , and R.G. Cook The novel SLIK histone acetyltransferase complex functions in the yeast retrograde response pathway Mol. Cell. Biol. 22 2002 8774 8786
71. D. Qi , J. Larsson , and M. Mannervik Drosophila Ada2b is required for viability and normal histone H3 acetylation Mol. Cell. Biol. 24 2004 8080 8089
72. K. Robzyk , J. Recht , and M.A. Osley Rad6-dependent ubiquitination of histone H2B in yeast Science 287 2000 501 504
73. S.Y. Roth , J.M. Denu , and C.D. Allis Histone acetyltransferases Annu. Rev. Biochem. 70 2001 81 120
74. G. Sadri-Vakili , B. Bouzou , C.L. Benn , M.O. Kim , P. Chawla , and R.P. Overland Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models Hum. Mol. Genet. 16 2007 1293 1306
75. S.L. Sanders , K.A. Garbett , and P.A. Weil Molecular characterization of Saccharomyces cerevisiae TFIID Mol. Cell. Biol. 22 2002 6000 6013
76. A. Shilatifard Chromatin modifications by methylation and ubiquitination: implications in the regulation of gene expression Annu. Rev. Biochem. 75 2006 243 269
77. M. Shogren-Knaak , H. Ishii , J.M. Sun , M.J. Pazin , J.R. Davie , and C.L. Peterson Histone H4-K16 acetylation controls chromatin structure and protein interactions Science 311 2006 844 847
78. A. Shukla , P. Bajwa , and S.R. Bhaumik SAGA-associated Sgf73p facilitates formation of the preinitiation complex assembly at the promoters either in a HAT-dependent or independent manner in vivo Nucleic Acids Res. 34 2006 6225 6232
79. E.R. Smith , J.M. Belote , R.L. Schiltz , X.J. Yang , P.A. Moore , and S.L. Berger Cloning of Drosophila GCN5: conserved features among metazoan GCN5 family members Nucleic Acids Res. 26 1998 2948 2954
80. K.T. Smith , and J.L. Workman Histone deacetylase inhibitors: anticancer compounds Int. J. Biochem. Cell Biol. 41 2009 21 25
81. J.S. Steffan , A. Kazantsev , O. Spasic-Boskovic , M. Greenwald , Y.Z. Zhu , and H. Gohler The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription Proc. Natl. Acad. Sci. U.S.A. 97 2000 6763 6768
82. D.E. Sterner , R. Belotserkovskaya , and S.L. Berger SALSA, a variant of yeast SAGA, contains truncated Spt7, which correlates with activated transcription Proc. Natl. Acad. Sci. U.S.A. 99 2002 11622 11627
83. B.D. Strahl , and C.D. Allis The language of covalent histone modifications Nature 403 2000 41 45
84. J.C. Strahlendorf , T. Brandon , R. Miles , and H.K. Strahlendorf AMPA receptor-mediated alterations of intracellular calcium homeostasis in rat cerebellar Purkinje cells in vitro: correlates to dark cell degeneration Neurochem. Res. 23 1998 1355 1362
85. J.C. Strahlendorf , and H.K. Strahlendorf Enduring changes in Purkinje cell electrophysiology following transient exposure to AMPA: correlates to dark cell degeneration Neurosci. Res. 33 1999 155 162
86. S.D. Taverna , H. Li , A.J. Ruthenburg , C.D. Allis , and D.J. Patel How chromatin-binding modules interpret histone modifications: lessons from professional pocket pickers Nat. Struct. Mol. Biol. 14 2007 1025 1040
87. B. Titz , S. Thomas , S.V. Rajagopala , T. Chiba , T. Ito , and P. Uetz Transcriptional activators in yeast Nucleic Acids Res. 34 2006 955 967
88. M.S. Torok , and P.A. Grant Histone acetyltransferase proteins contribute to transcriptional processes at multiple levels Adv. Protein Chem. 67 2004 181 199
89. P. Uetz , L. Giot , G. Cagney , T.A. Mansfield , R.S. Judson , and J.R. Knight A comprehensive analysis of protein-protein interactions in Saccharomyces cerevisiae Nature 403 2000 623 627
90. Y.L. Wang , F. Faiola , M. Xu , S. Pan , and E. Martinez Human ATAC Is a GCN5/PCAF-containing acetylase complex with a novel NC2-like histone fold module that interacts with the TATA-binding protein J. Biol. Chem. 283 2008 33808 33815
91. V.M. Weake , K.K. Lee , S. Guelman , C.H. Lin , C. Seidel , and S.M. Abmayr SAGA-mediated H2B deubiquitination controls the development of neuronal connectivity in the Drosophila visual system EMBO J. 27 2008 394 405
92. V.M. Weake , and J.L. Workman Histone ubiquitination: triggering gene activity Mol. Cell. 29 2008 653 663
93. C.L. Wellington , L.M. Ellerby , A.S. Hackam , R.L. Margolis , M.A. Trifiro , and R. Singaraja Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract J. Biol. Chem. 273 1998 9158 9167
94. A. Wyce , T. Xiao , K.A. Whelan , C. Kosman , W. Walter , and D. Eick H2B ubiquitylation acts as a barrier to Ctk1 nucleosomal recruitment prior to removal by Ubp8 within a SAGA-related complex Mol. Cell. 27 2007 275 288
95. W. Xu , D.G. Edmondson , Y.A. Evrard , M. Wakamiya , R.R. Behringer , and S.Y. Roth Loss of Gcn5l2 leads to increased apoptosis and mesodermal defects during mouse development Nat. Genet. 26 2000 229 232
96. W. Xu , D.G. Edmondson , and S.Y. Roth Mammalian GCN5 and P/CAF acetyltransferases have homologous amino-terminal domains important for recognition of nucleosomal substrates Mol. Cell. Biol. 18 1998 5659 5669
97. T. Yamauchi , J. Yamauchi , T. Kuwata , T. Tamura , T. Yamashita , and N. Bae Distinct but overlapping roles of histone acetylase PCAF and of the closely related PCAF-B/GCN5 in mouse embryogenesis Proc. Natl. Acad. Sci. U.S.A. 97 2000 11303 11306
98. X.J. Yang , V.V. Ogryzko , J. Nishikawa , B.H. Howard , and Y. Nakatani A p300/CBP-associated factor that competes with the adenoviral oncoprotein E1A Nature 382 1996 319 324
99. M. Ying , R. Xu , X. Wu , H. Zhu , Y. Zhuang , and M. Han Sodium butyrate ameliorates histone hypoacetylation and neurodegenerative phenotypes in a mouse model for DRPLA J. Biol. Chem. 281 2006 12580 12586
100. S.Y. Yoo , M.E. Pennesi , E.J. Weeber , B. Xu , R. Atkinson , and S. Chen SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity Neuron 37 2003 383 401
101. J.E. Young , L. Gouw , S. Propp , B.L. Sopher , J. Taylor , and A. Lin Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation J. Biol. Chem. 282 2007 30150 30160
102. G. Yvert , K.S. Lindenberg , D. Devys , D. Helmlinger , G.B. Landwehrmeyer , and J.L. Mandel SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types Hum. Mol. Genet. 10 2001 1679 1692
103. G. Yvert , K.S. Lindenberg , S. Picaud , G.B. Landwehrmeyer , J.A. Sahel , and J.L. Mandel Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice Hum. Mol. Genet. 9 2000 2491 2506
104. Y. Zhao , G. Lang , S. Ito , J. Bonnet , E. Metzger , and S. Sawatsubashi A TFTC/STAGA module mediates histone H2A and H2B deubiquitination, coactivates nuclear receptors, and counteracts heterochromatin silencing Mol. Cell. 29 2008 92 101