… mouse … mouse mAb against ATXN3 (1:1000; MAB 5360, Chemicon, Hampshire, UK), rabbit
pAb against Ub (1:600; Z 0458, DAKO, Hamburg, Germany), biotin labeled horse anti mouse (…

… mouse has not yet been created. The initial aim of this study was to generate an ataxin-3
humanized mouse model … To inactivate the mouse ataxin-3 gene, the strategy was designed to …

… In summary, overexpression of full-length mutant ataxin-3 in the mouse cerebellum using
LV induces neuropathological abnormalities and a robust motor behavior phenotype within 6 …

… (SNP) at the 3′ end of the CAG tract of the ataxin-3 gene is … This SNP can be used to
selectively inactivate mutant ataxin-3, … homology between mouse ataxin-3 and human ataxin-3 in …

… of ataxin-3 exon 9 and 10 in mouse To examine ataxin-3 exon skipping in the mouse brain
… harmful, we designed AONs specific to the mouse sequence. Since mice do not exhibit the …

… We focused on the question whether ataxin-3 is cleaved by calpain-1 or calpain-2 in mouse
brain in vivo and how this process would influence aggregate formation and neuropathology…

… inactivate mutant ataxin-3 selectively. Lentiviral-mediated silencing of mutant human ataxin-3
… with the appropriate antibodies—the mouse monoclonal anti-ataxin-3 antibody 1H9 (1∶…

Ataxin-3 (ATXN3) is a widely expressed protein that binds to ubiquitylated proteins, has … (siRNA)
causes a prominent phenotype in both human and mouse cell lines. A mild increase in …

… The transgene construct was prepared by inserting cDNA of ataxin-3-Q79HA or ataxin-3-Q22HA
into mouse prion protein expression vector (MoPrP.Xho) (Borchelt et al., 1996) …

… increase in DNA damage in mutant ataxin-3 cells, SCA3 mouse brain, and brain sections …
, could mutant ataxin-3 nonetheless interact with PNKP in its native complex to inactivate its …