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2 This appears to be the case with the protein ataxin 1 (ATXN1), which forms a transcriptional represso
7 reviously that partial suppression of mutant ataxin-1 (ATXN1) expression, using virally expressed RNA
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
23 escent cells, causing rapid decay of targets Ataxin-1 and Snurportin-1, and preventing premature sene
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
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
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
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
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
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
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.
70 gile X mental retardation protein (FMRP) and Ataxin-2 (Atx2) are triplet expansion disease- and stres
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
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
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
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
92 d by the conserved RNA-binding protein ATX-2/Ataxin-2, which targets and maintains ZEN-4 at the spind
95 f three members of the Josephin family DUBs: ataxin 3 (ATXN3), ataxin 3-like (ATXN3L) and Josephin do
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
105 the Josephin family DUBs: ataxin 3 (ATXN3), ataxin 3-like (ATXN3L) and Josephin domain containing 1
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
112 n whereby ubiquitination at Lys-117 enhances ataxin-3 activity independent of the known ubiquitin-bin
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
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
125 four proteins, Parkin acted on nNOS and Q78 ataxin-3 but not on the steroid receptors, and Mdm2 did
128 olyQ) repeat expansion in the deubiquitinase ataxin-3 causes neurodegeneration in Spinocerebellar Ata
132 bitor compound calpeptin decreased levels of ataxin-3 cleavage fragments, but also removed all human
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
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
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
159 ng and aggregation of the Josephin domain of ataxin-3 is implicated in spinocerebellar ataxia-3.
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
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
172 anio rerio) model of MJD by expressing human ataxin-3 protein containing either 23 glutamines (23Q, w
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
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
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.
193 Here we show that, unlike most proteins, ataxin-3 turnover does not require its ubiquitination, b
199 ssociated E3 ubiquitin-ligase interacts with ataxin-3, a deubiquitinating enzyme associated with Mach
201 uitination directly enhances the activity of ataxin-3, a DUb implicated in protein quality control an
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
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
218 ound in four human deubiquitinating enzymes: ataxin-3, the ataxin-3-like protein (ATXN3L), Josephin-1
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
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
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
256 subunit of the P/Q-type calcium channel, and ataxin-7 (ATXN7), a component of a chromatin-remodeling
258 ted or reversed SCA7 motor symptoms, reduced ataxin-7 aggregation in Purkinje cells (PCs), and preven
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
264 tion mouse model by inserting a loxP-flanked ataxin-7 cDNA with 92 repeats into the translational sta
270 molecular layer thinning, while excision of ataxin-7 from PCs and inferior olive provided significan
276 y reported that directed expression of polyQ-ataxin-7 in Bergmann glia (BG) in transgenic mice leads
279 When we examined the consequences of reduced Ataxin-7 in vivo, we found that flies exhibited pronounc
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
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
295 lts in increased H2B ubiquitination, loss of Ataxin-7 results in decreased H2B ubiquitination and H3K
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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