Literature for peptidase C86.001: ataxin-3

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

(Topics flags: S Structure, T Target, M Mutation, R Splicing, I Inhibitor, K Knockout, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

2024

Cheng,Y., Wang,S., Gao,Q. and Fang,D. ATXN3 functions as a tumor suppresser through potentiating Galectin-9-mediated apoptosis in human colon adenocarcinoma J Biol Chem (2024) , 107415-107415. PubMed Europe PubMed DOI
Felicio,D., Martins,S., Alves,G.P., Amorim,A., Macedo-Ribeiro,S. and Merski,M. Evolutionary model of repeat insertions in Ataxin-3 traces the origin of the polyglutamine stretch to an ancestral ubiquitin binding module Protein Sci (2024) 33, e5236-e5236. PubMed Europe PubMed DOI PMC EPMC
Olie,C.S., O'Brien,D.P., Jones,H.B.L., Liang,Z., Damianou,A., Sur-Erdem,I., Pinto-Fernandez,A., Raz,V. and Kessler,B.M. Deubiquitinases in muscle physiology and disorders Biochem Soc Trans (2024) 52, 1085-1098. PubMed Europe PubMed DOI V
Potapenko,A., Davidson,J.M., Lee,A. and Laird,A.S. The deubiquitinase function of ataxin-3 and its role in the pathogenesis of Machado-Joseph disease and other diseases Biochem J (2024) 481, 461-480. PubMed Europe PubMed DOI V
Stahl,F., Evert,B.O., Han,X., Breuer,P. and Wullner,U. Spinocerebellar Ataxia Type 3 Pathophysiology-Implications for Translational Research and Clinical Studies Int J Mol Sci (2024) 25 PubMed Europe PubMed DOI PMC EPMC

2023

Bai,J., Han,J., Fan,J., Song,J. and Wang,S. ATXN3 promotes proliferation, stemness and motility of clear cell renal cell carcinoma cells by regulating S100A8 ubiquitination Physiol Int (2023) 110, 311-325. PubMed Europe PubMed DOI
Liu,N., Lin,M.M. and Wang,Y. The Emerging Roles of E3 Ligases and DUBs in Neurodegenerative Diseases Mol Neurobiol (2023) 60, 247-263. PubMed Europe PubMed DOI PMC EPMC V
Melo,A.R.V., Raposo,M., Ventura,M., Martins,S., Pavao,S., Alonso,I., Bettencourt,C. and Lima,M. Genetic Variation in ATXN3 (Ataxin-3) 3'UTR: Insights into the Downstream Regulatory Elements of the Causative Gene of Machado-Joseph Disease/Spinocerebellar Ataxia Type 3 Cerebellum (2023) 22, 37-45. PubMed Europe PubMed DOI
Paulino,R. and Nobrega,C. Autophagy in Spinocerebellar Ataxia Type 3: From Pathogenesis to Therapeutics Int J Mol Sci (2023) 24 PubMed Europe PubMed DOI PMC EPMC V
Piasecki,P., Wiatr,K., Ruszkowski,M., Marczak,L., Trottier,Y. and Figiel,M. Impaired interactions of ataxin-3 with protein complexes reveals their specific structure and functions in SCA3 Ki150 model Front Mol Neurosci (2023) 16, 1122308-1122308. PubMed Europe PubMed DOI PMC EPMC
Wang,S., Liu,K., Han,X., Cheng,Y., Zhao,E., Brat,D.J., Sun,Z. and Fang,D. ATXN3 deubiquitinates YAP1 to promote tumor growth Am J Cancer Res (2023) 13, 4222-4234. PubMed Europe PubMed PMC EPMC
Wu,L., Ou,Z., Liu,P., Zhao,C., Tong,S., Wang,R., Li,Y., Yuan,J., Chen,M., Fan,B., Zu,X., Wang,Y. and Tang,J. ATXN3 promotes prostate cancer progression by stabilizing YAP Cell Commun Signal (2023) 21, 152-152. PubMed Europe PubMed DOI PMC EPMC
Wu,X., Zhang,X., Liu,P. and Wang,Y. Involvement of Ataxin-3 (ATXN3) in the malignant progression of pancreatic cancer via deubiquitinating HDAC6 Pancreatology (2023) 23, 630-641. PubMed Europe PubMed DOI K

2022

Johnson,S.L., Prifti,M.V., Sujkowski,A., Libohova,K., Blount,J.R., Hong,L., Tsou,W.L. and Todi,S.V. Drosophila as a Model of Unconventional Translation in Spinocerebellar Ataxia Type 3 Cells (2022) 11 PubMed Europe PubMed DOI PMC EPMC
Moharir,S.C., Raghawan,A.K., Ramaswamy,R. and Swarup,G. Autophagy-independent cytoprotection by optineurin from toxicity of aggregates formed by mutant huntingtin and mutant ataxin-3 J Biochem (2022) 171, 555-565. PubMed Europe PubMed DOI
Schuster,K.H., Zalon,A.J., Zhang,H., DiFranco,D.M., Stec,N.R., Haque,Z., Blumenstein,K.G., Pierce,A.M., Guan,Y., Paulson,H.L. and McLoughlin,H.S. Impaired Oligodendrocyte Maturation Is an Early Feature in SCA3 Disease Pathogenesis J Neurosci (2022) 42, 1604-1617. PubMed Europe PubMed DOI PMC EPMC

2021

Fardghassemi,Y., Maios,C. and Parker,J.A. Small Molecule Rescue of ATXN3 Toxicity in C. elegans via TFEB/HLH-30 Neurotherapeutics (2021) PubMed Europe PubMed DOI I
Pfeiffer,A., Herzog,L.K., Luijsterburg,M.S., Shah,R.G., Rother,M.B., Stoy,H., Kuhbacher,U., van Attikum,H., Shah,G.M. and Dantuma,N.P. Poly(ADP-ribosyl)ation temporally confines SUMO-dependent ataxin-3 recruitment to control DNA double-strand break repair J Cell Sci (2021) PubMed Europe PubMed DOI
Raj,K. and Akundi,R.S. Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder Mol Neurobiol (2021) PubMed Europe PubMed DOI V
Wrobel,L., Hill,S.M., Ashkenazi,A. and Rubinsztein,D.C. VCP/p97 modulates PtdIns3P production and autophagy initiation Autophagy (2021) 17, 1052-1053. PubMed Europe PubMed DOI
Yue,H.W., Hong,J.Y., Zhang,S.X., Jiang,L.L. and Hu,H.Y. PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates Sci Rep (2021) 11, 7815-7815. PubMed Europe PubMed DOI PMC EPMC
Zhang,Y., Shao,A.W., Tang,J. and Geng,Y. PML-II recruits ataxin-3 to PML-NBs and inhibits its deubiquitinating activity Biochem Biophys Res Commun (2021) 554, 186-192. PubMed Europe PubMed DOI
Zhuang,S., Xie,J., Zhen,J., Guo,L., Hong,Z., Li,F. and Xu,D. The deubiquitinating enzyme ATXN3 promotes the progression of anaplastic thyroid carcinoma by stabilizing EIF5A2 Mol Cell Endocrinol (2021) 537, 111440-111440. PubMed Europe PubMed DOI

2020

Dantuma,N.P. and Herzog,L.K. Machado-Joseph disease: a stress combating deubiquitylating enzyme changing sides Adv Exp Med Biol (2020) 1233, 237-260. PubMed Europe PubMed DOI M
Gerson,J.E., Safren,N., Fischer,S., Patel,R., Crowley,E.V., Welday,J.P., Windle,A.K., Barmada,S., Paulson,H.L. and Sharkey,L.M. Ubiquilin-2 differentially regulates polyglutamine disease proteins Hum Mol Genet (2020) PubMed Europe PubMed DOI
Jazurek-Ciesiolka,M., Ciesiolka,A., Komur,A.A., Urbanek-Trzeciak,M.O., Krzyzosiak,W.J. and Fiszer,A. RAN translation of the expanded CAG repeats in the SCA3 disease context J Mol Biol (2020) PubMed Europe PubMed DOI
Tu,Y., Li,X., Zhu,X., Liu,X., Guo,C., Jia,D. and Tang,T.S. Determining the Fate of Neurons in SCA3: ATX3, a Rising Decision Maker in Response to DNA Stresses and Beyond Front Cell Dev Biol (2020) 8, 619911-619911. PubMed Europe PubMed DOI PMC EPMC V
Yao,Y., Hong,S., Ikeda,T., Mori,H., MacDougald,O.A., Nada,S., Okada,M. and Inoki,K. Amino Acids Enhance Polyubiquitination of Rheb and Its Binding to mTORC1 by Blocking Lysosomal ATXN3 Deubiquitinase Activity Mol Cell (2020) 80, 437-451. PubMed Europe PubMed DOI PMC EPMC

2019

Herzog,L.K., Kevei,E., Marchante,R., Bottcher,C., Bindesboll,C., Lystad,A.H., Pfeiffer,A., Gierisch,M.E., Salomons,F.A., Simonsen,A., Hoppe,T. and Dantuma,N.P. The Machado-Joseph disease deubiquitylase ataxin-3 interacts with LC3C/GABARAP and promotes autophagy Aging Cell (2019) , e13051-e13051. PubMed Europe PubMed DOI
Nobrega,C., Codesso,J.M., Mendonca,L. and Pereira de Almeida,L. RNA interference therapy for Machado-Joseph disease: long-term safety profile of lentiviral vectors encoding short hairpin RNAs targeting mutant ataxin-3 Hum Gene Ther (2019) 30, 841-854. PubMed Europe PubMed DOI

2018

Contessotto,M.G.G., Rosselli-Murai,L.K., Garcia,M.C.C., Oliveira,C.L.P., Torriani,I.L., Lopes-Cendes,I. and Murai,M.J. The Machado-Joseph disease-associated expanded form of ataxin-3: overexpression, purification, and preliminary biophysical and structural characterization Protein Expr Purif (2018) 152, 40-45. PubMed Europe PubMed DOI
Feng,Q., Miao,Y., Ge,J., Yuan,Y., Zuo,Y., Qian,L., Liu,J., Cheng,Q., Guo,T., Zhang,L., Yu,Z. and Zheng,H. ATXN3 Positively regulates type I IFN antiviral response by deubiquitinating and stabilizing HDAC3 J Immunol (2018) 201, 675-687. PubMed Europe PubMed DOI
Sicorello,A., Kelly,G., Oregioni,A., Novacek,J., Sklenar,V. and Pastore,A. The structural properties in solution of the intrinsically mixed folded protein ataxin-3 Biophys J (2018) 115, 59-71. PubMed Europe PubMed DOI PMC EPMC S

2016

Deriu,M.A., Grasso,G., Tuszynski,J.A., Gallo,D., Morbiducci,U. and Danani,A. Josephin domain structural conformations explored by metadynamics in essential coordinates PLoS Comput Biol (2016) 12, e1004699-e1004699. PubMed Europe PubMed DOI PMC EPMC S
Liu,H., Li,X., Ning,G., Zhu,S., Ma,X., Liu,X., Liu,C., Huang,M., Schmitt,I., Wullner,U., Niu,Y., Guo,C., Wang,Q. and Tang,T.S. The Machado-Joseph disease deubiquitinase ataxin-3 regulates the stability and apoptotic function of p53 PLoS Biol (2016) 14, e2000733-e2000733. PubMed Europe PubMed DOI

2015

Faggiano,S., Menon,R.P., Kelly,G.P., Todi,S.V., Scaglione,K.M., Konarev,P.V., Svergun,D.I., Paulson,H.L. and Pastore,A. Allosteric regulation of deubiquitylase activity through ubiquitination Front Mol Biosci (2015) 2, 2- PubMed Europe PubMed DOI PMC EPMC
Pereira,R.V., de Souza Gomes,M., Costa,M.P., Jannotti Passos,L.K., de Castro Borges,W. and Guerra-Sa,R. MJD and OTU deubiquitinating enzymes in Schistosoma mansoni Parasitol Res (2015) 114, 2835-2843. PubMed Europe PubMed DOI
Pfoh,R., Lacdao,I.K. and Saridakis,V. Deubiquitinases and the new therapeutic opportunities offered to cancer Endocr Relat Cancer (2015) 22, T35-T54. PubMed Europe PubMed DOI V T

2014

Sanfelice,D., De Simone,A., Cavalli,A., Faggiano,S., Vendruscolo,M. and Pastore,A. Characterization of the conformational fluctuations in the Josephin domain of ataxin-3 Biophys J (2014) 107, 2932-2940. PubMed Europe PubMed DOI PMC EPMC
Satoh,T., Sumiyoshi,A., Yagi-Utsumi,M., Sakata,E., Sasakawa,H., Kurimoto,E., Yamaguchi,Y., Li,W., Joazeiro,C.A., Hirokawa,T. and Kato,K. Mode of substrate recognition by the Josephin domain of ataxin-3, which has an endo-type deubiquitinase activity FEBS Lett (2014) 588, 4422-4430. PubMed Europe PubMed DOI

2013

Durcan,T.M. and Fon,E.A. Ataxin-3 and its E3 partners: implications for Machado-Joseph disease Front Neurol (2013) 4, 46-46. PubMed Europe PubMed DOI PMC EPMC
Faggiano,S., Menon,R.P., Kelly,G.P., McCormick,J., Todi,S.V., Scaglione,K.M., Paulson,H.L. and Pastore,A. Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3 FEBS Open Bio (2013) 3, 453-458. PubMed Europe PubMed DOI PMC EPMC
Nobrega,C., Nascimento-Ferreira,I., Onofre,I., Albuquerque,D., Hirai,H., Deglon,N. and de Almeida,L.P. Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice PLoS ONE (2013) 8, e52396-e52396. PubMed Europe PubMed DOI PMC EPMC
Tsou,W.L., Burr,A.A., Ouyang,M., Blount,J.R., Scaglione,K.M. and Todi,S.V. Ubiquitination regulates the neuroprotective function of the deubiquitinase ataxin-3 in vivo J Biol Chem (2013) 288, 34460-34469. PubMed Europe PubMed DOI
Zeng,L., Tallaksen-Greene,S.J., Wang,B., Albin,R.L. and Paulson,H.L. The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease J Huntingtons Dis (2013) 2, 201-215. PubMed Europe PubMed DOI PMC EPMC

2012

Colomer Gould,V.F. Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease) Neurotherapeutics (2012) 9, 285-296. PubMed Europe PubMed DOI M
Durcan,T.M., Kontogiannea,M., Bedard,N., Wing,S.S. and Fon,E.A. Ataxin-3 deubiquitination is coupled to parkin ubiquitination via E2 ubiquitin-conjugating enzyme J Biol Chem (2012) 287, 531-541. PubMed Europe PubMed DOI
Todi,S.V., Pastore,A. and Paulson,H.L. Ataxin-3 [ISSN:978-0-12-407743-0] (2012) 3, 2123-2130. DOI

2011

Durcan,T.M., Kontogiannea,M., Thorarinsdottir,T., Fallon,L., Williams,A.J., Djarmati,A., Fantaneanu,T., Paulson,H.L. and Fon,E.A. The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability Hum Mol Genet (2011) 20, 141-154. PubMed Europe PubMed DOI M
Rodrigues,A.J., Neves-Carvalho,A., Teixeira-Castro,A., Rokka,A., Corthals,G., Logarinho,E. and Maciel,P. Absence of ataxin-3 leads to enhanced stress response in C. elegans PLoS ONE (2011) 6, e18512-e18512. PubMed Europe PubMed DOI PMC EPMC K
Scaglione,K.M., Zavodszky,E., Todi,S.V., Patury,S., Xu,P., Rodriguez-Lebron,E., Fischer,S., Konen,J., Djarmati,A., Peng,J., Gestwicki,J.E. and Paulson,H.L. Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP Mol Cell (2011) 43, 599-612. PubMed Europe PubMed DOI
Switonski,P.M., Fiszer,A., Kazmierska,K., Kurpisz,M., Krzyzosiak,W.J. and Figiel,M. Mouse ataxin-3 functional knock-out model Neuromolecular Med (2011) 13, 54-65. PubMed Europe PubMed DOI K M
Takeshita,Y., Fujinaga,R., Kokubu,K., Islam,M.N., Jahan,M.R., Yanai,A., Kakizuka,A. and Shinoda,K. Interaction of ataxin-3 with huntingtin-associated protein 1 through Josephin domain Neuroreport (2011) 22, 232-238. PubMed Europe PubMed DOI
Weeks,S.D., Grasty,K.C., Hernandez-Cuebas,L. and Loll,P.J. Crystal structure of a Josephin-ubiquitin complex: evolutionary restraints on ATAXIN-3 deubiquitinating activity J Biol Chem (2011) 286, 4555-4565. PubMed Europe PubMed DOI S

2010

Alves,S., Nascimento-Ferreira,I., Dufour,N., Hassig,R., Auregan,G., Nobrega,C., Brouillet,E., Hantraye,P., Pedroso de Lima,M.C., Deglon,N. and de Almeida,L.P. Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3? Hum Mol Genet (2010) 19, 2380-2394. PubMed Europe PubMed DOI K
Bettencourt,C., Santos,C., Montiel,R., Costa Mdo,C., Cruz-Morales,P., Santos,L.R., Simoes,N., Kay,T., Vasconcelos,J., Maciel,P. and Lima,M. Increased transcript diversity: novel splicing variants of Machado-Joseph disease gene (ATXN3) Neurogenetics (2010) 11, 193-202. PubMed Europe PubMed DOI M
do Carmo Costa,M., Bajanca,F., Rodrigues,A.J., Tome,R.J., Corthals,G., Macedo-Ribeiro,S., Paulson,H.L., Logarinho,E. and Maciel,P. Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels PLoS ONE (2010) 5, e11728-e11728. PubMed Europe PubMed DOI PMC EPMC
Harris,G.M., Dodelzon,K., Gong,L., Gonzalez-Alegre,P. and Paulson,H.L. Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties PLoS ONE (2010) 5, e13695-e13695. PubMed Europe PubMed DOI PMC EPMC R
Nicastro,G., Todi,S.V., Karaca,E., Bonvin,A.M., Paulson,H.L. and Pastore,A. Understanding the role of the Josephin domain in the polyUb binding and cleavage properties of ataxin-3 PLoS ONE (2010) 5, e12430-e12430. PubMed Europe PubMed DOI PMC EPMC
Reina,C.P., Zhong,X. and Pittman,R.N. Proteotoxic stress increases nuclear localization of ataxin-3 Hum Mol Genet (2010) 19, 235-249. PubMed Europe PubMed DOI
Rodrigues,A.J., do Carmo Costa,M., Silva,T.L., Ferreira,D., Bajanca,F., Logarinho,E. and Maciel,P. Absence of ataxin-3 leads to cytoskeletal disorganization and increased cell death Biochim Biophys Acta (2010) 1803, 1154-1163. PubMed Europe PubMed DOI
Song,A.X., Zhou,C.J., Peng,Y., Gao,X.C., Zhou,Z.R., Fu,Q.S., Hong,J., Lin,D.H. and Hu,H.Y. Structural transformation of the tandem ubiquitin-interacting motifs in ataxin-3 and their cooperative interactions with ubiquitin chains PLoS ONE (2010) 5, e13202-e13202. PubMed Europe PubMed DOI PMC EPMC
Todi,S.V., Scaglione,K.M., Blount,J.R., Basrur,V., Conlon,K.P., Pastore,A., Elenitoba-Johnson,K. and Paulson,H.L. Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117 J Biol Chem (2010) 285, 39303-39313. PubMed Europe PubMed DOI

2009

Antony,P.M., Mantele,S., Mollenkopf,P., Boy,J., Kehlenbach,R.H., Riess,O. and Schmidt,T. Identification and functional dissection of localization signals within ataxin-3 Neurobiol Dis (2009) 36, 280-292. PubMed Europe PubMed DOI
Mazzucchelli,S., De Palma,A., Riva,M., D'Urzo,A., Pozzi,C., Pastori,V., Comelli,F., Fusi,P., Vanoni,M., Tortora,P., Mauri,P. and Regonesi,M.E. Proteomic and biochemical analyses unveil tight interaction of ataxin-3 with tubulin Int J Biochem Cell Biol (2009) 41, 2485-2492. PubMed Europe PubMed DOI
Nicastro,G., Masino,L., Esposito,V., Menon,R.P., De Simone,A., Fraternali,F. and Pastore,A. Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites Biopolymers (2009) 91, 1203-1214. PubMed Europe PubMed DOI
Rodrigues,A.J., Neves-Carvalho,A., Ferro,A., Rokka,A., Corthals,G., Logarinho,E. and Maciel,P. ATX-3, CDC-48 and UBXN-5: A new trimolecular complex in Caenorhabditis elegans Biochem Biophys Res Commun (2009) 386, 575-581. PubMed Europe PubMed DOI
Todi,S.V., Winborn,B.J., Scaglione,K.M., Blount,J.R., Travis,S.M. and Paulson,H.L. Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3 EMBO J (2009) 28, 372-382. PubMed Europe PubMed DOI PMC EPMC

2008

Pozzi,C., Valtorta,M., Tedeschi,G., Galbusera,E., Pastori,V., Bigi,A., Nonnis,S., Grassi,E. and Fusi,P. Study of subcellular localization and proteolysis of ataxin-3 Neurobiol Dis (2008) 30, 190-200. PubMed Europe PubMed DOI
Winborn,B.J., Travis,S.M., Todi,S.V., Scaglione,K.M., Xu,P., Williams,A.J., Cohen,R.E., Peng,J. and Paulson,H.L. The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains J Biol Chem (2008) 283, 26436-26443. PubMed Europe PubMed DOI PMC EPMC

2007

Bichelmeier,U., Schmidt,T., Hubener,J., Boy,J., Ruttiger,L., Habig,K., Poths,S., Bonin,M., Knipper,M., Schmidt,W.J., Wilbertz,J., Wolburg,H., Laccone,F. and Riess,O. Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence J Neurosci (2007) 27, 7418-7428. PubMed Europe PubMed DOI
Colomer Gould,V.F., Goti,D., Pearce,D., Gonzalez,G.A., Gao,H., Bermudez de Leon,M., Jenkins,N.A., Copeland,N.G., Ross,C.A. and Brown,D.R. A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice Neurobiol Dis (2007) 27, 362-369. PubMed Europe PubMed DOI PMC EPMC M
Ellisdon,A.M., Pearce,M.C. and Bottomley,S.P. Mechanisms of ataxin-3 misfolding and fibril formation: kinetic analysis of a disease-associated polyglutamine protein J Mol Biol (2007) 368, 595-605. PubMed Europe PubMed DOI
Rodrigues,A.J., Coppola,G., Santos,C., Costa,M., Ailion,M., Sequeiros,J., Geschwind,D.H. and Maciel,P. Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3 FASEB J (2007) 21, 1126-1136. PubMed Europe PubMed DOI
Schmitt,I., Linden,M., Khazneh,H., Evert,B.O., Breuer,P., Klockgether,T. and Wuellner,U. Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination Biochem Biophys Res Commun (2007) 362, 734-739. PubMed Europe PubMed DOI K
Todi,S.V., Laco,M.N., Winborn,B.J., Travis,S.M., Wen,H.M. and Paulson,H.L. Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity J Biol Chem (2007) 282, 29348-29358. PubMed Europe PubMed DOI

2006

Ellisdon,A.M., Thomas,B. and Bottomley,S.P. The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step J Biol Chem (2006) 281, 16888-16896. PubMed Europe PubMed DOI
Evert,B.O., Araujo,J., Vieira-Saecker,A.M., de Vos,R.A., Harendza,S., Klockgether,T. and Wullner,U. Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation J Neurosci (2006) 26, 11474-11486. PubMed Europe PubMed DOI
Wang,Q., Li,L. and Ye,Y. Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3 J Cell Biol (2006) 174, 963-971. PubMed Europe PubMed DOI PMC EPMC
Zhong,X. and Pittman,R.N. Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates Hum Mol Genet (2006) 15, 2409-2420. PubMed Europe PubMed DOI

2005

Berke,S.J., Chai,Y., Marrs,G.L., Wen,H. and Paulson,H.L. Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3 J Biol Chem (2005) 280, 32026-32034. PubMed Europe PubMed DOI
Burnett,B.G. and Pittman,R.N. The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation Proc Natl Acad Sci U S A (2005) 102, 4330-4335. PubMed Europe PubMed DOI PMC EPMC
Mao,Y., Senic-Matuglia,F., Di Fiore,P.P., Polo,S., Hodsdon,M.E. and De Camilli,P. Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain Proc Natl Acad Sci U S A (2005) 102, 12700-12705. PubMed Europe PubMed DOI PMC EPMC
[YEAR:14-7-2005]Nicastro,G., Menon,R.P., Masino,L., Knowles,P.P., McDonald,N.Q. and Pastore,A. The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition Proc Natl Acad Sci U S A (14-7-2005) 102, 10493-10498. PubMed Europe PubMed DOI PMC EPMC
[YEAR:1-4-2005]Warrick,J.M., Morabito,L.M., Bilen,J., Gordesky-Gold,B., Faust,L.Z., Paulson,H.L. and Bonini,N.M. Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism Mol Cell (1-4-2005) 18, 37-48. PubMed Europe PubMed DOI

2004

Albrecht,M., Golatta,M., Wullner,U. and Lengauer,T. Structural and functional analysis of ataxin-2 and ataxin-3 Eur J Biochem (2004) 271, 3155-3170. PubMed Europe PubMed DOI
Chai,Y., Berke,S.S., Cohen,R.E. and Paulson,H.L. Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways J Biol Chem (2004) 279, 3605-3611. PubMed Europe PubMed DOI
[YEAR:17-9-2004]Chow,M.K., Mackay,J.P., Whisstock,J.C., Scanlon,M.J. and Bottomley,S.P. Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3 Biochem Biophys Res Commun (17-9-2004) 322, 387-394. PubMed Europe PubMed DOI
Nicastro,G., Masino,L., Frenkiel,T.A., Kelly,G., McCormick,J., Menon,R.P. and Pastore,A. Assignment of the 1H, 13C, and 15N resonances of the Josephin domain of human ataxin-3 J Biomol NMR (2004) 30, 457-458. PubMed Europe PubMed DOI S

2003

[YEAR:1-12-2003]Burnett,B., Li,F. and Pittman,R.N. The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity Hum Mol Genet (1-12-2003) 12, 3195-3205. PubMed Europe PubMed DOI
Doss-Pepe,E.W., Stenroos,E.S., Johnson,W.G. and Madura,K. Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis Mol Cell Biol (2003) 23, 6469-6483. PubMed Europe PubMed DOI PMC EPMC
[YEAR:1-11-2003]Scheel,H., Tomiuk,S. and Hofmann,K. Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics Hum Mol Genet (1-11-2003) 12, 2845-2852. PubMed Europe PubMed DOI

2002

Li,F., Macfarlan,T., Pittman,R.N. and Chakravarti,D. Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities J Biol Chem (2002) 277, 45004-45012. PubMed Europe PubMed DOI

1998

Schmidt,T., Landwehrmeyer,G.B., Schmitt,I., Trottier,Y., Auburger,G., Laccone,F., Klockgether,T., Volpel,M., Epplen,J.T., Schols,L. and Riess,O. An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients Brain Pathol (1998) 8, 669-679. PubMed Europe PubMed M

1997

Paulson,H.L., Das,S.S., Crino,P.B., Perez,M.K., Patel,S.C., Gotsdiner,D., Fischbeck,K.H. and Pittman,R.N. Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain Ann Neurol (1997) 41, 453-462. PubMed Europe PubMed DOI

1994

Kawaguchi,Y., Okamoto,T., Taniwaki,M., Aizawa,M., Inoue,M., Katayama,S., Kawakami,H., Nakamura,S., Nishimura,M. and Akiguchi,I. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1 Nat Genet (1994) 8, 221-228. PubMed Europe PubMed DOI