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1                                              Ataxin 1 (ATXN1) is one of these four AD candidate genes
2 This appears to be the case with the protein ataxin 1 (ATXN1), which forms a transcriptional represso
3 sed by expansion of a polyglutamine tract in ataxin 1 (ATXN1).
4  expansion of a glutamine-encoding repeat in ataxin 1 (ATXN1).
5 DP-43 itself, FUS/TLS, progranulin, Tau, and ataxin 1 and -2.
6       We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegene
7 reviously that partial suppression of mutant ataxin-1 (ATXN1) expression, using virally expressed RNA
8 xpansion of a polyglutamine tract within the ataxin-1 (ATXN1) protein.
9 , the expanded polyglutamine tract is in the ataxin-1 (ATXN1) protein.
10        To further investigate the ability of ataxin-1 (ATXN1) to impact CF/PC innervation, this study
11 d by expansion of a translated CAG repeat in Ataxin-1 (ATXN1).
12 CAG repeat encoding a polyglutamine tract in Ataxin-1 (ATXN1).
13  by expansion of a glutamine repeat tract in ataxin-1 (ATXN1).
14 ic glutamine repeat expansion in the protein ataxin-1 (ATXN1).
15 ansmission required expression of pathogenic ataxin-1 (ATXN1[82Q]) and for its entrance into the nucl
16 genic mice that overexpress the normal human ataxin-1 (the SCA1[30Q] line) and wild-type controls.
17 genic mice that overexpress the mutant human ataxin-1 (the SCA1[82Q] line) were measured longitudinal
18  as a cellular model to assess stress due to ataxin-1 82Q protein expression and determine whether NP
19                            Furthermore, when ataxin-1 82Q was expressed in 15-lipoxygenase-1-deficien
20                                Essential for ataxin-1 aggregation is the anomalous expansion of a pol
21 thology are phosphorylation of serine 776 in Ataxin-1 and nuclear localization of the protein.
22                              Using wild-type Ataxin-1 and Ser776 mutants to a phosphomimetic aspartat
23 escent cells, causing rapid decay of targets Ataxin-1 and Snurportin-1, and preventing premature sene
24  their respective ubiquitination substrates, Ataxin-1 and Snurportin-1.
25  relevance for the aggregation properties of ataxin-1 and thus for disease.
26 -3-3 interacts with phosphorylated wild-type Ataxin-1 but not with the mutants.
27 er, HOTAIR facilitates the ubiquitination of Ataxin-1 by Dzip3 and Snurportin-1 by Mex3b in cells and
28 nomalous expansion of a polymorphic tract in Ataxin-1 causes the autosomal dominant spinocerebellar a
29 ling modulates formation or stabilization of ataxin-1 complexes.
30                                      Ectopic ataxin-1 expression induced RPE cell apoptosis, which wa
31 city induced by mutant huntingtin and mutant ataxin-1 expression.
32 survival through modulating stabilization of ataxin-1 functional complexes and pro-/antiapoptotic and
33 te and to alanine, we show that U2AF65 binds Ataxin-1 in a Ser776 phosphorylation independent manner
34 gene expression and decreased phosphorylated ataxin-1 in an Akt-independent manner, suggesting that N
35                                              Ataxin-1 is a human protein responsible for spinocerebel
36                       Evidence suggests that ataxin-1 may function as a transcription repressor.
37 ine 776 is also crucial for selection of the Ataxin-1 multiple partners.
38  inclusions formed by polyglutamine-expanded ataxin-1 or huntingtin.
39 T for an in cell study of the interaction of Ataxin-1 with the spliceosome-associated U2AF65 and the
40  proteotoxic stress due to abnormally folded ataxin-1, and 2) NPD1 promotes cell survival through mod
41 related proteins (polyglutamine, huntingtin, ataxin-1, and superoxide dismutase-1) inhibits clathrin-
42  polyglutamine disease proteins (huntingtin, ataxin-1, ataxin-7 and androgen receptor) via polyglutam
43  investigated whether polyglutamine-expanded ATAXIN-1, the protein that underlies spinocerebellar ata
44                       NPD1 reduced misfolded ataxin-1-induced accumulation of proapoptotic Bax in the
45 ing that sacsin is protective against mutant ataxin-1.
46 d sacsin knockdown on polyglutamine-expanded ataxin-1.
47      Finally, NPD1 signaling interfered with ataxin-1/capicua repression of gene expression and decre
48 siRNA did not affect cell viability with GFP-ataxin-1[30Q], but enhanced the toxicity of GFP-ataxin-1
49 xin-1[30Q], but enhanced the toxicity of GFP-ataxin-1[82Q], suggesting that sacsin is protective agai
50 functional DUE or at the recently identified ataxin 10 (ATX10) origin, which is silent before disease
51                            Here we show that ataxin 2 (ATXN2), a polyglutamine (polyQ) protein mutate
52 isposing to ALS and that polyQ expansions in ataxin 2 are a significant risk factor for the disease.
53 lyglutamine (polyQ) expansions (27-33 Qs) in ataxin 2 as a genetic risk factor for sporadic ALS in No
54 h antineoplastic assay and identified A2BP1 (ataxin 2 binding protein 1, Rbfox1), an RNA-binding and
55                        Repeats of CAG in the ataxin 2 gene (ATXN2) in the long-normal range (sometime
56                                              Ataxin 2 intermediate-length polyglutamine (polyQ) expan
57 results provide mechanistic insight into how ataxin 2 intermediate-length polyQ expansions could cont
58 BP1, are a known ALS genetic risk factor and ataxin 2 is a stress granule component in mammalian cell
59     Our findings support the hypothesis that ataxin 2 plays an important role in predisposing to ALS
60                               Because longer ataxin 2 polyQ expansions are associated with a differen
61       Here, we show that intermediate-length ataxin 2 polyQ expansions enhance stress-induced TDP-43
62          We also connect intermediate-length ataxin 2 polyQ expansions to the stress-dependent activa
63 triking association with ALS cases harboring ataxin 2 polyQ expansions.
64 ical feature of ALS with intermediate-length ataxin 2 polyQ expansions.
65                    Thus, intermediate-length ataxin 2 polyQ repeat expansions are associated with inc
66    To extend these findings, we assessed the ataxin 2 polyQ repeat length in 1294 European ALS patien
67  we report functional analysis of Drosophila Ataxin 2-binding protein 1 (A2BP1) during this process.
68 pends on the Drosophila homolog of the human ataxin 2-binding protein 1 (A2BP1) gene.
69                           We show that Fox-1/Ataxin 2-Binding Protein 1 (A2BP1), a protein implicated
70 gile X mental retardation protein (FMRP) and Ataxin-2 (Atx2) are triplet expansion disease- and stres
71                                We found that ATAXIN-2 (ATX2), an RNA-associated protein involved in n
72      We found that the Drosophila homolog of ATAXIN-2 (ATX2)--an RNA-binding protein implicated in hu
73 lutamine) expansion in the cytosolic protein ataxin-2 (Atx2).
74 olyglutamine tracts in the cytosolic protein ataxin-2 (Atx2).
75                                              Ataxin-2 (ATXN2) homologs exist in all eukaryotic organi
76            Expanded glutamine repeats of the ataxin-2 (ATXN2) protein cause spinocerebellar ataxia ty
77 ing the binding of 2 of its client proteins, ataxin-2 and Sf3b2.
78 oteins implicated in neurodegeneration (i.e. Ataxin-2 and SMN) interact with stress granules.
79 ndians (n = 413) identified variation in the ataxin-2 binding protein 1 gene (A2BP1) that was associa
80 ponent of nearly all cases of ALS, targeting ataxin-2 could represent a broadly effective therapeutic
81 diate-length polyglutamine expansions in the ataxin-2 gene increase risk of ALS.
82 -43, FUS (fused in sarcoma), angiogenin, and ataxin-2 in amyotrophic lateral sclerosis; ataxin-2 in s
83 d ataxin-2 in amyotrophic lateral sclerosis; ataxin-2 in spinocerebellar ataxia; and SMN (survival of
84                            First, we crossed ataxin-2 knockout mice with TDP-43 (also known as TARDBP
85  this association and the obese phenotype of ataxin-2 knockout mice, A2BP1 was genetically and functi
86 endent approaches to test whether decreasing ataxin-2 levels could mitigate disease in a mouse model
87                              The decrease in ataxin-2 reduced aggregation of TDP-43, markedly increas
88                                A decrease in ataxin-2 suppresses TDP-43 toxicity in yeast and flies,
89 ble approach, we administered ASOs targeting ataxin-2 to the central nervous system of TDP-43 transge
90 the levels of two client proteins (SF3B2 and ataxin-2) of a chaperone protein, heat shock protein 90
91 ns associated with ALS, including TDP-43 and ataxin-2, is that they localize to stress granules.
92 d by the conserved RNA-binding protein ATX-2/Ataxin-2, which targets and maintains ZEN-4 at the spind
93 ues, similar to the expression of endogenous ataxin-2.
94 tic strategy for ALS that involves targeting ataxin-2.
95 f three members of the Josephin family DUBs: ataxin 3 (ATXN3), ataxin 3-like (ATXN3L) and Josephin do
96                             This activity of ataxin 3 and its polyQ-mediated interaction with beclin
97 ch as huntingtin in Huntington's disease and ataxin 3 in spinocerebellar ataxia type 3 (SCA3).
98 her polyQ disease proteins, including mutant ataxin 3 itself.
99 5 mice with citalopram significantly reduced ataxin 3 neuronal inclusions and astrogliosis, rescued d
100 1 and SER-4 were strong genetic modifiers of ataxin 3 neurotoxicity and necessary for therapeutic eff
101 show that the polyQ domain enables wild-type ataxin 3 to interact with beclin 1, a key initiator of a
102 action allows the deubiquitinase activity of ataxin 3 to protect beclin 1 from proteasome-mediated de
103  such protein is the deubiquitinating enzyme ataxin 3, which is widely expressed in the brain.
104 ing a Caenorhabditis elegans model of mutant ataxin 3-induced neurotoxicity.
105  the Josephin family DUBs: ataxin 3 (ATXN3), ataxin 3-like (ATXN3L) and Josephin domain containing 1
106 ne mRNA allele encoding huntingtin (HTT) and ataxin-3 (ATX-3) proteins.
107 rom expansion of the polyglutamine domain in ataxin-3 (Atx3).
108 order caused by a polyglutamine expansion in ataxin-3 (ATX3; MJD1) protein.
109 eads to misfolding and aggregation of mutant ataxin-3 (ATXN3) and degeneration of select brain region
110 expanded CAG repeats within an allele of the ataxin-3 (ATXN3) and huntingtin (HTT) genes, respectivel
111                The physiological function of Ataxin-3 (ATXN3), a deubiquitylase (DUB) involved in Mac
112 n whereby ubiquitination at Lys-117 enhances ataxin-3 activity independent of the known ubiquitin-bin
113 crease local structural fluctuations to slow ataxin-3 aggregation.
114      For example, polyglutamine expansion in ataxin-3 allosterically triggers the aggregation of the
115                                     Although ataxin-3 and ATXN3L adopt similar folds, they bind ubiqu
116 AG repeats can preferentially inhibit mutant ataxin-3 and HTT protein expression in cultured cells.
117 ophagy pathway prevents the removal of human ataxin-3 and improved movement produced by calpeptin tre
118  pathways mediated by polyglutamine-expanded ataxin-3 and that phosphorylation of this residue protec
119                     These findings establish ataxin-3 as a novel DUB that edits topologically complex
120  also increased the levels of polyQ-expanded ataxin-3 as well as mutant alpha-synuclein and superoxid
121 icate that ubiquitin-dependent activation of ataxin-3 at Lys-117 is important for its ability to redu
122 re, we report that ubiquitination of the DUB ataxin-3 at lysine residue 117, which markedly enhances
123                       Mutations were made in ataxin-3 at selected positions, introducing the correspo
124                                              Ataxin-3 binds both Lys(48)- or Lys(63)-linked chains ye
125  four proteins, Parkin acted on nNOS and Q78 ataxin-3 but not on the steroid receptors, and Mdm2 did
126                                  The protein ataxin-3 carries a polyglutamine stretch close to the C-
127                                       Mutant ataxin-3 caused an evolving neuronal dysfunction (loss o
128 olyQ) repeat expansion in the deubiquitinase ataxin-3 causes neurodegeneration in Spinocerebellar Ata
129            Polyglutamine repeat expansion in ataxin-3 causes neurodegeneration in the most common dom
130  caspase-site mutant proteins indicates that Ataxin-3 cleavage enhances neuronal loss in vivo.
131 n-3-23Q and develop ataxin-3 neuropathology, ataxin-3 cleavage fragments and motor impairment.
132 bitor compound calpeptin decreased levels of ataxin-3 cleavage fragments, but also removed all human
133    However, the pathological significance of Ataxin-3 cleavage has not been carefully examined.
134 ivo confirmation of the pathological role of Ataxin-3 cleavage indicates that therapies targeting Ata
135  cleavage indicates that therapies targeting Ataxin-3 cleavage might slow disease progression in SCA3
136     To gain insight into the significance of Ataxin-3 cleavage, we developed a Drosophila SL2 cell-ba
137                                              Ataxin-3 cleaves ubiquitin chains through its amino-term
138  a potential target through which to enhance ataxin-3 degradation for SCA3 therapy.
139 bility of two juxtaposed helices critical to ataxin-3 deubiquitinase activity.
140 Upon completion of substrate ubiquitination, ataxin-3 deubiquitinates CHIP, effectively terminating t
141 in and onto ataxin-3, further explaining how ataxin-3 deubiquitination is coupled to parkin ubiquitin
142 he strain expressing full-length, functional ataxin-3 displayed persistent upregulation of enzymes in
143 significantly more efficient enzyme than the ataxin-3 domain despite their sharing 85% sequence ident
144 nt to increase the catalytic activity of the ataxin-3 domain to levels comparable with that of ATXN3L
145 ed in the yeast Pichia pastoris, full-length ataxin-3 enabled almost normal growth at 37 degrees C, w
146 ss-siRNAs are allele-selective inhibitors of ataxin-3 expression and then redesign ss-siRNAs to optim
147 anisms that protect Josephin and nonexpanded ataxin-3 from aberrant aggregation.
148 ting the autoprotective role that pathogenic ataxin-3 has against itself, which depends on the co-cha
149 parable with that of ATXN3L, suggesting that ataxin-3 has been subject to evolutionary restraints tha
150 mice with lentiviral vectors encoding mutant ataxin-3 in one hemisphere and wild-type ataxin-3 in the
151 d, suggesting that the cellular functions of ataxin-3 in protein quality control are modulated throug
152 ant ataxin-3 in one hemisphere and wild-type ataxin-3 in the other hemisphere (as internal control).
153 ), an E3 ubiquitin ligase that ubiquitinates ataxin-3 in vitro, is dispensable for its ubiquitination
154 ) associated with increased number of mutant ataxin-3 inclusions in the basal ganglia.
155       In cells, ubiquitination of endogenous ataxin-3 increases when the proteasome is inhibited, whe
156                                              Ataxin-3 interacts with the proteasome-associated protei
157                          We report here that ataxin-3 interferes with the attachment of ubiquitin (Ub
158 es pathology in animals, we investigated how ataxin-3 is degraded.
159 ng and aggregation of the Josephin domain of ataxin-3 is implicated in spinocerebellar ataxia-3.
160                        We report that S12 of ataxin-3 is phosphorylated in neurons and that mutating
161                                              Ataxin-3 is the first reported DUB in which ubiquitinati
162 or chloroquine blocked the decrease in human ataxin-3 levels and the improved movement produced by ca
163 activity in wild-type mice but not in orexin/ataxin-3 mice in which the Hcrt neurons degenerate postn
164 ranscripts that were decreased in transgenic ataxin-3 mice that were normalized following temsirolimu
165                                       Orexin/ataxin-3 mice, in which the Hcrt neurons degenerate, did
166 vival compared with ataxin-3-23Q and develop ataxin-3 neuropathology, ataxin-3 cleavage fragments and
167  suggest that functional pairing of E3s with ataxin-3 or similar DUBs represents an important point o
168  other modifiers of the pathogenic, expanded Ataxin-3 polyQ protein could also modify the CAG-repeat
169 eavage fragments, but also removed all human ataxin-3 protein (confirmed by ELISA) and prevented the
170         We identified that this clearance of ataxin-3 protein by calpeptin treatment resulted from an
171                       Our data indicate that Ataxin-3 protein cleavage is conserved in the fly and ma
172 anio rerio) model of MJD by expressing human ataxin-3 protein containing either 23 glutamines (23Q, w
173  6 days old, even if expression of the human ataxin-3 protein is limited to motor neurons.
174                      Mutating UbS2 decreases ataxin-3 protein levels in cultured mammalian cells and
175                                              Ataxin-3 protein with an expanded polyglutamine (polyQ)
176 calpeptin produces complete removal of human ataxin-3 protein, due to induction of the autophagy qual
177  that induction of autophagy, and removal of ataxin-3 protein, plays an important role in the protect
178 anded polyglutamine (polyQ) tract within the Ataxin-3 protein.
179 f the polyglutamine repeat region within the ataxin-3 protein.
180 lating into a polyglutamine tract within the ataxin-3 protein.
181 and in Drosophila results in lower levels of ataxin-3 protein.
182                                              Ataxin-3 shows even greater activity toward mixed linkag
183                  Our work also suggests that ataxin-3 suppresses degeneration by regulating toxic pro
184 red with ataxin-3 with only Lys-117 present, ataxin-3 that does not become ubiquitinated performs sig
185 ve in this model of MJD and removal of human ataxin-3 through macro-autophagy plays an important role
186 n at Lys-117 also facilitates the ability of ataxin-3 to induce aggresome formation in cells.
187 y of a number of proteins that interact with Ataxin-3 to modulate SCA3 pathogenicity using Drosophila
188 e compared to wild-type mice, whereas orexin/ataxin-3 transgenic mice showed an intermediate 28% incr
189  these systems in 6 wild-type mice, 6 orexin/ataxin-3 transgenic mice, and 5 orexin ligand knockout m
190  was delivered into the brains of the orexin-ataxin-3 transgenic mouse model of human narcolepsy.
191 ockout mice and orexin neuron-ablated orexin/ataxin-3 transgenic rats.
192                              Notably, mutant ataxin-3 triggered early synaptotoxicity (decreased syna
193     Here we show that, unlike most proteins, ataxin-3 turnover does not require its ubiquitination, b
194                                              Ataxin-3 was found to counteract parkin self-ubiquitinat
195      In vitro studies revealed that inactive ataxin-3 was more slowly degraded by the proteasome and
196                     Unexpectedly, pathogenic ataxin-3 with a mutated Rad23-binding site at UbS2, desp
197                                Compared with ataxin-3 with only Lys-117 present, ataxin-3 that does n
198                                              Ataxin-3's degradation is inhibited by its binding to th
199 ssociated E3 ubiquitin-ligase interacts with ataxin-3, a deubiquitinating enzyme associated with Mach
200                                              Ataxin-3, a deubiquitinating enzyme, is the disease prot
201 uitination directly enhances the activity of ataxin-3, a DUb implicated in protein quality control an
202                         We further show that ataxin-3, a p97-associated deubiquitinating enzyme previ
203 so directly regulates the activity of a DUB, ataxin-3, a polyglutamine disease protein implicated in
204 disease (MJD), also known as spinocerebellar ataxin-3, affects neurons of the brain and spinal cord,
205 parkin required the catalytic cysteine 14 in ataxin-3, although the precise mechanism remained unclea
206 substrate, the polyglutamine disease protein ataxin-3, and showed that Ube2w can ubiquitinate a lysin
207 show that the polyglutamine disease protein, ataxin-3, binds and cleaves ubiquitin chains in a manner
208 on enhances ubiquitin (Ub) chain cleavage by ataxin-3, but does not alter its preference for K63-link
209 ermining solubility and aggregation rates of ataxin-3, but these properties are profoundly modulated
210 s Rad23 increases the toxicity of pathogenic ataxin-3, coincident with increased levels of the diseas
211 e N terminus of unmodified and ubiquitinated ataxin-3, demonstrating that Ube2w attaches ubiquitin to
212                                     Expanded ataxin-3, for example, is more neurotoxic in fruit fly m
213 the E2 is diverted away from parkin and onto ataxin-3, further explaining how ataxin-3 deubiquitinati
214  Finally, expression of the disease protein, ataxin-3, in transfected cells increases the inactivatio
215 dent of the known ubiquitin-binding sites in ataxin-3, most likely through a direct conformational ch
216 e specialized deubiquitinating enzyme (DUB), ataxin-3, participate in initiating, regulating, and ter
217 rom the activity of deubiquitinases, such as ataxin-3, that are necessary for efficient ERAD.
218 ound in four human deubiquitinating enzymes: ataxin-3, the ataxin-3-like protein (ATXN3L), Josephin-1
219                                       Mutant ataxin-3, the genetic cause of Machado-Joseph Disease, a
220                           Here, we show that ataxin-3, the protein involved in spinocerebellar ataxia
221                                              Ataxin-3, the protein responsible for Spinocerebellar at
222  implications for the function of parkin and ataxin-3, two proteins responsible for closely related n
223 spinocerebellar ataxia type 3 (SCA3) protein ataxin-3, we isolated an upregulation allele of musclebl
224  stabilizes the interaction between CHIP and ataxin-3, which through its DUB activity limits the leng
225 decreases levels of cytosolic soluble mutant ataxin-3, while endogenous wild-type protein levels rema
226 rafish have decreased survival compared with ataxin-3-23Q and develop ataxin-3 neuropathology, ataxin
227 -3-84Q zebrafish swim shorter distances than ataxin-3-23Q zebrafish as early as 6 days old, even if e
228 ebrafish (male and female) revealed that the ataxin-3-84Q zebrafish have decreased survival compared
229                                              Ataxin-3-84Q zebrafish swim shorter distances than ataxi
230                            Treating the EGFP-ataxin-3-84Q zebrafish with the calpain inhibitor compou
231       Importantly, reducing Rad23 suppresses ataxin-3-dependent degeneration in flies.
232                                    Moreover, ataxin-3-dependent deubiquitination of parkin required t
233                    The mechanism involves an ataxin-3-dependent stabilization of the complex between
234 d by beclin 1, was particularly inhibited in ataxin-3-depleted human cell lines and mouse primary neu
235 ld(S) VCP-binding domain with an alternative ataxin-3-derived VCP-binding sequence restores its prote
236  rats stereotaxically injected with expanded ataxin-3-encoding lentiviral vectors, mutation of serine
237 uman deubiquitinating enzymes: ataxin-3, the ataxin-3-like protein (ATXN3L), Josephin-1, and Josephin
238  narcolepsy models: Hcrt (orexin) knockouts, ataxin-3-orexin, and doxycycline-controlled-diphtheria-t
239 we tested whether genetically modulating the ataxin-3-Rad23 interaction regulates its toxicity in Dro
240 nd potentially therapeutic properties of the ataxin-3-Rad23 interaction; they highlight this interact
241                   We found that adult orexin/ataxin-3-transgenic (AT) mice, in which Hcrt neurons deg
242 a lysine-less, but not N-terminally blocked, ataxin-3.
243  into an expanded polyglutamine tract within ataxin-3.
244  situ by aggregate-associated deubiquitinase ataxin-3.
245 isorder caused by polyglutamine expansion in ataxin-3.
246 f ubiquitination in wild type and pathogenic ataxin-3.
247 e molecular mechanism whereby this occurs in ataxin-3.
248 K48-specific avidity in a different protein, ataxin-3.
249 o-expressed with the GR, nNOS, AR112Q or Q78 ataxin-3.
250 rupting this interaction decreases levels of ataxin-3.
251 lyubiquitin chains by the Josephin domain of ataxin-3.
252  be used to silence the endogenous allele of ataxin 7 and replace it with an exogenous copy of the ge
253 RNAs, and introduce silent mutations into an ataxin 7 transgene such that it is resistant to their ef
254             Here we develop mirtrons against ataxin 7 with silencing efficacy comparable to shRNAs, a
255  results from polyglutamine expansion of the ataxin-7 (ATXN7) protein.
256 subunit of the P/Q-type calcium channel, and ataxin-7 (ATXN7), a component of a chromatin-remodeling
257  to determine whether and how polyQ-expanded ataxin-7 affects SAGA catalytic activity.
258 ted or reversed SCA7 motor symptoms, reduced ataxin-7 aggregation in Purkinje cells (PCs), and preven
259                 Within this modular complex, Ataxin-7 anchors the deubiquitinase activity to the larg
260 mine disease proteins (huntingtin, ataxin-1, ataxin-7 and androgen receptor) via polyglutamine sequen
261 e we identified and characterized Drosophila Ataxin-7 and found that reduction of Ataxin-7 protein re
262                    Proteolytic processing of ataxin-7 by caspase-7 generates N-terminal toxic polyQ-c
263                                  Cleavage of ataxin-7 by the protease caspase-7 has been demonstrated
264 tion mouse model by inserting a loxP-flanked ataxin-7 cDNA with 92 repeats into the translational sta
265      Here, we determined that polyQ-expanded ataxin-7 directly bound the Gcn5 catalytic core of SAGA
266       Despite this phenotype rescue, reduced ataxin-7 expression did not result in full recovery of c
267                          To inactivate polyQ-ataxin-7 expression in specific cerebellar cell types, w
268 a, which resulted in ~50% reduction of polyQ-ataxin-7 expression.
269                                  Excision of ataxin-7 from BG partially rescued the behavioral phenot
270  molecular layer thinning, while excision of ataxin-7 from PCs and inferior olive provided significan
271                   Given this, we studied how ataxin-7 gene expression is regulated.
272             To understand how CTCF regulates ataxin-7 gene expression, we introduced ataxin-7 mini-ge
273 olyglutamine (polyQ) repeat expansion in the ataxin-7 gene.
274 olyglutamine (polyQ) repeat expansion in the ataxin-7 gene.
275 y CAG/polyglutamine repeat expansions in the ataxin-7 gene.
276 y reported that directed expression of polyQ-ataxin-7 in Bergmann glia (BG) in transgenic mice leads
277       When we prevented expression of mutant ataxin-7 in BG, PCs, and inferior olive by deriving Gfa2
278 quitinase module is active in the absence of Ataxin-7 in vitro.
279 When we examined the consequences of reduced Ataxin-7 in vivo, we found that flies exhibited pronounc
280          In contrast to yeast, where loss of Ataxin-7 inactivates the deubiquitinase and results in i
281                                              Ataxin-7 is a component of two different transcription c
282 cumulation of the fragment, while unmodified ataxin-7 is degraded.
283                              Modification of ataxin-7 K257 by acetylation promotes accumulation of th
284 at inhibition of caspase-7 cleavage of polyQ-ataxin-7 may be a promising therapeutic strategy for thi
285 ates ataxin-7 gene expression, we introduced ataxin-7 mini-genes into mice, and found that CTCF is re
286  modifications of the N-terminal fragment of ataxin-7 modulate turnover and toxicity.
287 -7 cleavage site is an important mediator of ataxin-7 neurotoxicity, suggesting that inhibition of ca
288 sophila Ataxin-7 and found that reduction of Ataxin-7 protein results in loss of components from the
289 xpansion within the N-terminal region of the ataxin-7 protein, a known subunit of the SAGA complex.
290   Polyglutamine (polyQ) expansion within the ataxin-7 protein, a member of the STAGA [SPT3-TAF(II)31-
291  by a polyglutamine (polyQ) expansion in the ataxin-7 protein, categorizing SCA7 as one member of a l
292 whether a causal relationship exists between ataxin-7 proteolysis and in vivo SCA7 disease progressio
293 d adjacent to the caspase-7 cleavage site of ataxin-7 regulates turnover of the truncation product in
294                                          The ataxin-7 repeat and translation start site are flanked b
295 lts in increased H2B ubiquitination, loss of Ataxin-7 results in decreased H2B ubiquitination and H3K
296                  Loss of SCAANT1 derepressed ataxin-7 sense transcription in a cis-dependent fashion
297 ed transgenic mice expressing polyQ-expanded ataxin-7 with a second-site mutation (D266N) to prevent
298 ork have altered expression in the retina of Ataxin-7(266Q/+) mice suggesting an in vivo functional r
299 ant-negative phenotype of the polyQ-expanded ataxin-7-incorporated, catalytically inactive SAGA.
300 ful suppressor of Wallerian degeneration and ataxin- and tau-induced neurodegeneration in flies and m

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