Journal: Cell reports
Article Title: Repeat-associated non-AUG translation as a common mechanism for the polyGln ataxias
doi: 10.1016/j.celrep.2025.116741
Figure Lengend Snippet: (A) IHC images show pontine polySer RAN aggregates increase in number and intensity with age in ATXN3 Q84/WT mice. (B) Quantification of polySer RAN aggregates in pons of non-transgenic and ATXN3 Q84/WT mice (* p < 0.025, two-tailed unpaired Student’s t test corrected for multiple comparisons). (C) Pcp2-ATXN1[82Q] mice, designed to express the transgene in Purkinje cells, show the expected polyGln signal in Purkinje cells, and surprisingly, also both sense polySer and antisense polyLeu RAN proteins throughout the cerebellum. Cerebellar regions showing positive RAN polySer and polyLeu staining show increased numbers of microglial cells. (D) Pcp2-82Q/V591A-S602D mice with reduced pathology and disruption of ATXN1-CIC interaction show decreased polySer, polyLeu, and Iba1 staining without changes in polyGln signal. (E) Quantification of polySer, polyLeu, polyGln, and Iba1 across cerebellar regions in non-transgenic, Pcp2-82Q, and Pcp2-82Q/V591A-S602D mice (* p < 0.05, two-tailed unpaired Student’s t test for single comparisons). (F) Schematic of a revised model of toxicity in Pcp2-82Q mice. Graphs show mean ± SEM from IHC quantification (average of 3 independent images per mouse; n = 4 per group for SCA3 YACQ84 and non-transgenic littermates; n = 3 per group for Pcp2-82Q, Pcp2-82Q-V591A-S602D, and non-transgenic littermates. MCL, molecular cell layer; BG, Bergmann glia; PCL, Purkinje cell layer; GCL, granular cell layer; WM, white matter. Red, positive staining; purple, nuclear counterstain. Black scale bars, 20 μm; blue scale bars, 5 μm. See also .
Article Snippet: Mouse: SCA3 YAC, ATXN3 wt/Q84 (MJD84.2. B6;CBA-Tg(ATXN3*)84.2Cce/IbezJ) , The Jackson Laboratory , JAX #012705; RRID: IMSR_JAX:012705.
Techniques: Transgenic Assay, Two Tailed Test, Staining, Disruption
Addgene inc is a verified supplier 
![(A) IHC images show pontine polySer RAN aggregates increase in number and intensity with age in <t>ATXN3</t> <t>Q84/WT</t> mice. (B) Quantification of polySer RAN aggregates in pons of non-transgenic and ATXN3 Q84/WT mice (* p < 0.025, two-tailed unpaired Student’s t test corrected for multiple comparisons). (C) Pcp2-ATXN1[82Q] mice, designed to express the transgene in Purkinje cells, show the expected polyGln signal in Purkinje cells, and surprisingly, also both sense polySer and antisense polyLeu RAN proteins throughout the cerebellum. Cerebellar regions showing positive RAN polySer and polyLeu staining show increased numbers of microglial cells. (D) Pcp2-82Q/V591A-S602D mice with reduced pathology and disruption of ATXN1-CIC interaction show decreased polySer, polyLeu, and Iba1 staining without changes in polyGln signal. (E) Quantification of polySer, polyLeu, polyGln, and Iba1 across cerebellar regions in non-transgenic, Pcp2-82Q, and Pcp2-82Q/V591A-S602D mice (* p < 0.05, two-tailed unpaired Student’s t test for single comparisons). (F) Schematic of a revised model of toxicity in Pcp2-82Q mice. Graphs show mean ± SEM from IHC quantification (average of 3 independent images per mouse; n = 4 per group for SCA3 YACQ84 and non-transgenic littermates; n = 3 per group for Pcp2-82Q, Pcp2-82Q-V591A-S602D, and non-transgenic littermates. MCL, molecular cell layer; BG, Bergmann glia; PCL, Purkinje cell layer; GCL, granular cell layer; WM, white matter. Red, positive staining; purple, nuclear counterstain. Black scale bars, 20 μm; blue scale bars, 5 μm. See also .](https://pub-med-central-images-cdn.bioz.com/pub_med_central_ids_ending_with_1278/pmc13131278/pmc13131278__nihms-2142933-f0007.jpg)