polyglutamine

ataxin 3 ; Mus musculus







64 Article(s)
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51 19699305 A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats. 2010 Feb 3
52 20007218 Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3. 2010 Jan 1
53 20510362 Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products. 2010 Oct 2
54 19084066 In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. 2009 Mar 4
55 18502140 Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation. 2008 Jul 4
56 18668148 PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner. 2008 Aug 10
57 17626202 Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence. 2007 Jul 11 4
58 17764659 Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination. 2007 Oct 26 2
59 16389311 Mouse models of Machado-Joseph disease and other polyglutamine spinocerebellar ataxias. 2005 Jul 2
60 15537899 A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration. 2004 Nov 10 2
61 11978767 YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit. 2002 May 1 1
62 12127147 Chemical chaperones reduce aggregate formation and cell death caused by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. 2002 Jul 5
63 11394998 Genetic ablation of orexin neurons in mice results in narcolepsy, hypophagia, and obesity. 2001 May 1
64 9020849 Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. 1997 Feb 2