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2. Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease. Simões AT; Gonçalves N; Koeppen A; Déglon N; Kügler S; Duarte CB; Pereira de Almeida L Brain; 2012 Aug; 135(Pt 8):2428-39. PubMed ID: 22843411 [TBL] [Abstract][Full Text] [Related]
3. RNA Interference Therapy for Machado-Joseph Disease: Long-Term Safety Profile of Lentiviral Vectors Encoding Short Hairpin RNAs Targeting Mutant Ataxin-3. Nóbrega C; Codêsso JM; Mendonça L; Pereira de Almeida L Hum Gene Ther; 2019 Jul; 30(7):841-854. PubMed ID: 30760052 [TBL] [Abstract][Full Text] [Related]
4. Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease. Nascimento-Ferreira I; Santos-Ferreira T; Sousa-Ferreira L; Auregan G; Onofre I; Alves S; Dufour N; Colomer Gould VF; Koeppen A; Déglon N; Pereira de Almeida L Brain; 2011 May; 134(Pt 5):1400-15. PubMed ID: 21478185 [TBL] [Abstract][Full Text] [Related]
5. Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease. Carmona V; Cunha-Santos J; Onofre I; Simões AT; Vijayakumar U; Davidson BL; Pereira de Almeida L Mol Ther; 2017 Apr; 25(4):1038-1055. PubMed ID: 28236575 [TBL] [Abstract][Full Text] [Related]
6. Toward therapeutic targets for SCA3: Insight into the role of Machado-Joseph disease protein ataxin-3 in misfolded proteins clearance. Li X; Liu H; Fischhaber PL; Tang TS Prog Neurobiol; 2015 Sep; 132():34-58. PubMed ID: 26123252 [TBL] [Abstract][Full Text] [Related]
7. Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease. Alves S; Régulier E; Nascimento-Ferreira I; Hassig R; Dufour N; Koeppen A; Carvalho AL; Simões S; de Lima MC; Brouillet E; Gould VC; Déglon N; de Almeida LP Hum Mol Genet; 2008 Jul; 17(14):2071-83. PubMed ID: 18385100 [TBL] [Abstract][Full Text] [Related]
9. Caffeine and adenosine A(2A) receptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado-Joseph disease. Gonçalves N; Simões AT; Cunha RA; de Almeida LP Ann Neurol; 2013 May; 73(5):655-66. PubMed ID: 23625556 [TBL] [Abstract][Full Text] [Related]
10. Neuropeptide Y mitigates neuropathology and motor deficits in mouse models of Machado-Joseph disease. Duarte-Neves J; Gonçalves N; Cunha-Santos J; Simões AT; den Dunnen WF; Hirai H; Kügler S; Cavadas C; Pereira de Almeida L Hum Mol Genet; 2015 Oct; 24(19):5451-63. PubMed ID: 26220979 [TBL] [Abstract][Full Text] [Related]
11. Ibuprofen enhances synaptic function and neural progenitors proliferation markers and improves neuropathology and motor coordination in Machado-Joseph disease models. Mendonça LS; Nóbrega C; Tavino S; Brinkhaus M; Matos C; Tomé S; Moreira R; Henriques D; Kaspar BK; Pereira de Almeida L Hum Mol Genet; 2019 Nov; 28(22):3691-3703. PubMed ID: 31127937 [TBL] [Abstract][Full Text] [Related]
12. Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease. Nascimento-Ferreira I; Nóbrega C; Vasconcelos-Ferreira A; Onofre I; Albuquerque D; Aveleira C; Hirai H; Déglon N; Pereira de Almeida L Brain; 2013 Jul; 136(Pt 7):2173-88. PubMed ID: 23801739 [TBL] [Abstract][Full Text] [Related]
13. The Machado-Joseph disease-associated expanded form of ataxin-3: Overexpression, purification, and preliminary biophysical and structural characterization. Contessotto MGG; Rosselli-Murai LK; Garcia MCC; Oliveira CLP; Torriani IL; Lopes-Cendes I; Murai MJ Protein Expr Purif; 2018 Dec; 152():40-45. PubMed ID: 30036587 [TBL] [Abstract][Full Text] [Related]
14. Altered glucose metabolism and its association with carbonic anhydrase 8 in Machado-Joseph Disease. Lin GY; Ma CY; Kuo LC; Hsieh BY; Wang H; Liu CS; Hsieh M Metab Brain Dis; 2022 Aug; 37(6):2103-2120. PubMed ID: 35488942 [TBL] [Abstract][Full Text] [Related]
15. Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado-Joseph disease. Simões AT; Gonçalves N; Nobre RJ; Duarte CB; Pereira de Almeida L Hum Mol Genet; 2014 Sep; 23(18):4932-44. PubMed ID: 24817574 [TBL] [Abstract][Full Text] [Related]
16. 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. Colomer Gould VF; Goti D; Pearce D; Gonzalez GA; Gao H; Bermudez de Leon M; Jenkins NA; Copeland NG; Ross CA; Brown DR Neurobiol Dis; 2007 Sep; 27(3):362-9. PubMed ID: 17632007 [TBL] [Abstract][Full Text] [Related]
17. Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: conclusions from double-transgenic mouse models. Hübener J; Riess O Neurobiol Dis; 2010 Apr; 38(1):116-24. PubMed ID: 20079840 [TBL] [Abstract][Full Text] [Related]
18. Unbiased screen identifies aripiprazole as a modulator of abundance of the polyglutamine disease protein, ataxin-3. Costa MDC; Ashraf NS; Fischer S; Yang Y; Schapka E; Joshi G; McQuade TJ; Dharia RM; Dulchavsky M; Ouyang M; Cook D; Sun D; Larsen MJ; Gestwicki JE; Todi SV; Ivanova MI; Paulson HL Brain; 2016 Nov; 139(11):2891-2908. PubMed ID: 27645800 [TBL] [Abstract][Full Text] [Related]
19. Identification of the calpain-generated toxic fragment of ataxin-3 protein provides new avenues for therapy of Machado-Joseph disease| Spinocerebellar ataxia type 3. Simões AT; Carmona V; Duarte-Neves J; Cunha-Santos J; Pereira de Almeida L Neuropathol Appl Neurobiol; 2022 Feb; 48(1):e12748. PubMed ID: 34273111 [TBL] [Abstract][Full Text] [Related]
20. A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3. Weber JJ; Golla M; Guaitoli G; Wanichawan P; Hayer SN; Hauser S; Krahl AC; Nagel M; Samer S; Aronica E; Carlson CR; Schöls L; Riess O; Gloeckner CJ; Nguyen HP; Hübener-Schmid J Brain; 2017 May; 140(5):1280-1299. PubMed ID: 28334907 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]