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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
  1. 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 Chem107415-107415. PubMed  Europe PubMed DOI
  2. 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 Sci33, e5236-e5236. PubMed  Europe PubMed DOI
  3. 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 Trans52, 1085-1098. PubMed  Europe PubMed DOI  V
  4. 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 J481, 461-480. PubMed  Europe PubMed DOI  V
  5. 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 Sci25, PubMed  Europe PubMed DOI
    6. 2023
  6. 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 Int110, 311-325. PubMed  Europe PubMed DOI
  7. Liu,N., Lin,M.M. and Wang,Y.
    The Emerging Roles of E3 Ligases and DUBs in Neurodegenerative Diseases
    Mol Neurobiol60, 247-263. PubMed  Europe PubMed DOI  V
  8. 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
    Cerebellum22, 37-45. PubMed  Europe PubMed DOI
  9. Paulino,R. and Nobrega,C.
    Autophagy in Spinocerebellar Ataxia Type 3: From Pathogenesis to Therapeutics
    Int J Mol Sci24, PubMed  Europe PubMed DOI  V
  10. 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 Neurosci16, 1122308-1122308. PubMed  Europe PubMed DOI
  11. 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 Res13, 4222-4234. PubMed  Europe PubMed
  12. 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 Signal21, 152-152. PubMed  Europe PubMed DOI
  13. Wu,X., Zhang,X., Liu,P. and Wang,Y.
    Involvement of Ataxin-3 (ATXN3) in the malignant progression of pancreatic cancer via deubiquitinating HDAC6
    Pancreatology23, 630-641. PubMed  Europe PubMed DOI  K
    14. 2022
  14. 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
    Cells11, PubMed  Europe PubMed DOI
  15. 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 Biochem171, 555-565. PubMed  Europe PubMed DOI
  16. 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 Neurosci42, 1604-1617. PubMed  Europe PubMed DOI
    17. 2021
  17. Fardghassemi,Y., Maios,C. and Parker,J.A.
    Small Molecule Rescue of ATXN3 Toxicity in C. elegans via TFEB/HLH-30
    Neurotherapeutics PubMed  Europe PubMed DOI  I
  18. 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 PubMed  Europe PubMed DOI
  19. Raj,K. and Akundi,R.S.
    Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder
    Mol Neurobiol PubMed  Europe PubMed DOI  V
  20. Wrobel,L., Hill,S.M., Ashkenazi,A. and Rubinsztein,D.C.
    VCP/p97 modulates PtdIns3P production and autophagy initiation
    Autophagy17, 1052-1053. PubMed  Europe PubMed DOI
  21. 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 Rep11, 7815-7815. PubMed  Europe PubMed DOI
  22. 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 Commun554, 186-192. PubMed  Europe PubMed DOI
  23. 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 Endocrinol537, 111440-111440. PubMed  Europe PubMed DOI
    24. 2020
  24. Dantuma,N.P. and Herzog,L.K.
    Machado-Joseph disease: a stress combating deubiquitylating enzyme changing sides
    Adv Exp Med Biol1233, 237-260. PubMed  Europe PubMed DOI  M
  25. 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 PubMed  Europe PubMed DOI
  26. 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 PubMed  Europe PubMed DOI
  27. 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 Biol8, 619911-619911. PubMed  Europe PubMed DOI  V
  28. 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 Cell80, 437-451. PubMed  Europe PubMed DOI
    29. 2019
  29. 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 Celle13051-e13051. PubMed  Europe PubMed DOI
  30. 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 Ther30, 841-854. PubMed  Europe PubMed DOI
    31. 2018
  31. 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 Purif152, 40-45. PubMed  Europe PubMed DOI
  32. 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 Immunol201, 675-687. PubMed  Europe PubMed DOI
  33. 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 J115, 59-71. PubMed  Europe PubMed DOI  S
    34. 2016
  34. 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 Biol12, e1004699-e1004699. PubMed  Europe PubMed DOI  S
  35. 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 Biol14, e2000733-e2000733. PubMed  Europe PubMed DOI
    36. 2015
  36. 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 Biosci2, 2- PubMed  Europe PubMed DOI
  37. 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 Res114, 2835-2843. PubMed  Europe PubMed DOI
  38. Pfoh,R., Lacdao,I.K. and Saridakis,V.
    Deubiquitinases and the new therapeutic opportunities offered to cancer
    Endocr Relat Cancer22, T35-T54. PubMed  Europe PubMed DOI  V  T
    39. 2014
  39. 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 J107, 2932-2940. PubMed  Europe PubMed DOI
  40. 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 Lett588, 4422-4430. PubMed  Europe PubMed DOI
    41. 2013
  41. Durcan,T.M. and Fon,E.A.
    Ataxin-3 and its E3 partners: implications for Machado-Joseph disease
    Front Neurol4, 46-46. PubMed  Europe PubMed DOI
  42. 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 Bio3, 453-458. PubMed  Europe PubMed DOI
  43. 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 ONE8, e52396-e52396. PubMed  Europe PubMed DOI
  44. 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 Chem288, 34460-34469. PubMed  Europe PubMed DOI
  45. 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 Dis2, 201-215. PubMed  Europe PubMed DOI
    46. 2012
  46. Colomer Gould,V.F.
    Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease)
    Neurotherapeutics9, 285-296. PubMed  Europe PubMed DOI  M
  47. 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 Chem287, 531-541. PubMed  Europe PubMed DOI
  48. Todi,S.V., Pastore,A. and Paulson,H.L.
    Ataxin-3
    [ISSN:978-0-12-407743-0]3, 2123-2130. DOI
    49. 2011
  49. 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 Genet20, 141-154. PubMed  Europe PubMed DOI  M
  50. 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 ONE6, e18512-e18512. PubMed  Europe PubMed DOI  K
  51. 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 Cell43, 599-612. PubMed  Europe PubMed DOI
  52. Switonski,P.M., Fiszer,A., Kazmierska,K., Kurpisz,M., Krzyzosiak,W.J. and Figiel,M.
    Mouse ataxin-3 functional knock-out model
    Neuromolecular Med13, 54-65. PubMed  Europe PubMed DOI  K  M
  53. 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
    Neuroreport22, 232-238. PubMed  Europe PubMed DOI
  54. 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 Chem286, 4555-4565. PubMed  Europe PubMed DOI  S
    55. 2010
  55. 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 Genet19, 2380-2394. PubMed  Europe PubMed DOI  K
  56. 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)
    Neurogenetics11, 193-202. PubMed  Europe PubMed DOI  M
  57. 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 ONE5, e11728-e11728. PubMed  Europe PubMed DOI
  58. 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 ONE5, e13695-e13695. PubMed  Europe PubMed DOI  R
  59. 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 ONE5, e12430-e12430. PubMed  Europe PubMed DOI
  60. Reina,C.P., Zhong,X. and Pittman,R.N.
    Proteotoxic stress increases nuclear localization of ataxin-3
    Hum Mol Genet19, 235-249. PubMed  Europe PubMed DOI
  61. 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 Acta1803, 1154-1163. PubMed  Europe PubMed DOI
  62. 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 ONE5, e13202-e13202. PubMed  Europe PubMed DOI
  63. 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 Chem285, 39303-39313. PubMed  Europe PubMed DOI
    64. 2009
  64. 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 Dis36, 280-292. PubMed  Europe PubMed DOI
  65. 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 Biol41, 2485-2492. PubMed  Europe PubMed DOI
  66. 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
    Biopolymers91, 1203-1214. PubMed  Europe PubMed DOI
  67. 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 Commun386, 575-581. PubMed  Europe PubMed DOI
  68. 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 J28, 372-382. PubMed  Europe PubMed DOI
    69. 2008
  69. 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 Dis30, 190-200. PubMed  Europe PubMed DOI
  70. 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 Chem283, 26436-26443. PubMed  Europe PubMed DOI
    71. 2007
  71. 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 Neurosci27, 7418-7428. PubMed  Europe PubMed DOI
  72. 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 Dis27, 362-369. PubMed  Europe PubMed DOI  M
  73. 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 Biol368, 595-605. PubMed  Europe PubMed DOI
  74. 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 J21, 1126-1136. PubMed  Europe PubMed DOI
  75. 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 Commun362, 734-739. PubMed  Europe PubMed DOI  K
  76. 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 Chem282, 29348-29358. PubMed  Europe PubMed DOI
    77. 2006
  77. Ellisdon,A.M., Thomas,B. and Bottomley,S.P.
    The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step
    J Biol Chem281, 16888-16896. PubMed  Europe PubMed DOI
  78. 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 Neurosci26, 11474-11486. PubMed  Europe PubMed DOI
  79. Wang,Q., Li,L. and Ye,Y.
    Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3
    J Cell Biol174, 963-971. PubMed  Europe PubMed DOI
  80. Zhong,X. and Pittman,R.N.
    Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates
    Hum Mol Genet15, 2409-2420. PubMed  Europe PubMed DOI
    81. 2005
  81. 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 Chem280, 32026-32034. PubMed  Europe PubMed DOI
  82. Burnett,B.G. and Pittman,R.N.
    The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation
    Proc Natl Acad Sci U S A102, 4330-4335. PubMed  Europe PubMed DOI
  83. 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 A102, 12700-12705. PubMed  Europe PubMed DOI
  84. [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 A102, 10493-10498. PubMed  Europe PubMed DOI
  85. [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 Cell18, 37-48. PubMed  Europe PubMed DOI
    86. 2004
  86. Albrecht,M., Golatta,M., Wullner,U. and Lengauer,T.
    Structural and functional analysis of ataxin-2 and ataxin-3
    Eur J Biochem271, 3155-3170. PubMed  Europe PubMed DOI
  87. 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 Chem279, 3605-3611. PubMed  Europe PubMed DOI
  88. [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 Commun322, 387-394. PubMed  Europe PubMed DOI
  89. 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 NMR30, 457-458. PubMed  Europe PubMed DOI  S
    90. 2003
  90. [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 Genet12, 3195-3205. PubMed  Europe PubMed DOI
  91. 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 Biol23, 6469-6483. PubMed  Europe PubMed DOI
  92. [YEAR:1-11-2003]Scheel,H., Tomiuk,S. and Hofmann,K.
    Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics
    Hum Mol Genet12, 2845-2852. PubMed  Europe PubMed DOI
    93. 2002
  93. 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 Chem277, 45004-45012. PubMed  Europe PubMed DOI
    94. 1998
  94. 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 Pathol8, 669-679. PubMed  Europe PubMed  M
    95. 1997
  95. 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 Neurol41, 453-462. PubMed  Europe PubMed DOI
    96. 1994
  96. 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 Genet8, 221-228. PubMed  Europe PubMed DOI

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