Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence
U Bichelmeier, T Schmidt, J Hübener, J Boy… - Journal of …, 2007 - Soc Neuroscience
… using full-length ataxin-3 constructs. We demonstrate in vivo that nuclear localization of
ataxin-3 is required for the generation of symptoms and that export of ataxin-3 out of the nucleus …
ataxin-3 is required for the generation of symptoms and that export of ataxin-3 out of the nucleus …
Proteotoxic stress increases nuclear localization of ataxin-3
CP Reina, X Zhong, RN Pittman - Human molecular genetics, 2010 - academic.oup.com
… required for nuclear localization of the Josephin domain and regulated nuclear localization
of … Importantly, we found that oxidative stress also induced nuclear localization of Atx3; both …
of … Importantly, we found that oxidative stress also induced nuclear localization of Atx3; both …
Heterogeneous intracellular localization and expression of ataxin-3
Y Trottier, G Cancel, I An-Gourfinkel, Y Lutz… - Neurobiology of …, 1998 - Elsevier
… A putative nuclear localization signal (NLS) (RKRR) is … is involved in the ataxin-3 nuclear
localization found in the Hela … the nuclear ataxin-3 is associated with the inner nuclear matrix …
localization found in the Hela … the nuclear ataxin-3 is associated with the inner nuclear matrix …
Identification and functional dissection of localization signals within ataxin-3
PMA Antony, S Mäntele, P Mollenkopf, J Boy… - Neurobiology of …, 2009 - Elsevier
… localization of ataxin-3 for the pathogenesis of SCA3 in vivo using transgenic mouse models.
We showed that nuclear localization of ataxin-3 … and that cytoplasmic ataxin-3 was not able …
We showed that nuclear localization of ataxin-3 … and that cytoplasmic ataxin-3 was not able …
Study of subcellular localization and proteolysis of ataxin-3
C Pozzi, M Valtorta, G Tedeschi, E Galbusera… - Neurobiology of …, 2008 - Elsevier
… or nuclear localization signal. Moreover, fragment 1 from AT-Q72 (27-FSPVE) was found
to be located in all cell compartments analyzed: (cytoplasm, nucleus, membranes, and …
to be located in all cell compartments analyzed: (cytoplasm, nucleus, membranes, and …
Ataxin-3 is transported into the nucleus and associates with the nuclear matrix
D Tait, M Riccio, A Sittler, E Scherzinger… - Human molecular …, 1998 - academic.oup.com
… is associated with the inner nuclear matrix. Our results taken together with the finding of a
nuclear localization signal in ataxin-3 indicate that the ataxin-3 protein per se translocates to …
nuclear localization signal in ataxin-3 indicate that the ataxin-3 protein per se translocates to …
CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3
T Mueller, P Breuer, I Schmitt, J Walter… - Human molecular …, 2009 - academic.oup.com
… nuclear localization, aggregation and stability of ataxin-3 (ATXN3), the disease protein in
spinocerebellar ataxia type 3 (… were particularly important for nuclear localization of normal and …
spinocerebellar ataxia type 3 (… were particularly important for nuclear localization of normal and …
Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado–Joseph …
AT Simoes, N Gonçalves, A Koeppen, N Deglon… - Brain, 2012 - academic.oup.com
… proteolysis by calpains is required for nuclear localization of mutant ataxin 3; (ii) inhibition of
… ; (iii) production of mutant ataxin 3 cleavage fragments and the resulting nuclear localization …
… ; (iii) production of mutant ataxin 3 cleavage fragments and the resulting nuclear localization …
Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3
AS Sowa, E Martin, IM Martins… - Proceedings of the …, 2018 - National Acad Sciences
… truncated and full-length expanded ataxin-3, rescue neurodegeneration, restore photoreceptor
… nuclear localization signals (NLSs), this work unites ataxin-3 structure to the nuclear pore …
… nuclear localization signals (NLSs), this work unites ataxin-3 structure to the nuclear pore …
[HTML][HTML] Nucleocytoplasmic shuttling activity of ataxin-3
… Since pathological Atx3 aggregates in the nucleus of affected neurons in MJD, and there
is in vivo evidence that nuclear localization of Atx3 is required for the manifestation of …
is in vivo evidence that nuclear localization of Atx3 is required for the manifestation of …
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