Figure 3.

ATXN2-luc expression in vivo was elevated in the cerebellum and OB compared with endogenous ATXN2. (A) For most tissues, the average expression of ATXN2-luc to endogenous mouse Atxn2 was 1:1 by qPCR. ATXN2-luc expression was high in some mice in the cerebellum and OB relative to mouse Atxn2 but the average ratio in the cerebellum was not significantly different from that for other tissues. qPCRs were run in triplicate and the reported values represent the means among mice ± SD. (B–E) Evaluation of ATXN2-luc expression on the IVIS. (B) ATXN2-luc expression in the nose (N) and cauda epithelium (CE) of a ATXN2-luc transgenic mouse. (C) Detection of ATXN2-luc in the OB and cerebellum (CB) in a 7-month-old ATXN2-luc transgenic mouse (TG) and lack of signal in a wild-type littermate (WT). (D) ATXN2-luc detected in the OB and CB of a 2-month-old ATXN2-luc transgenic mouse and lack of signal in a wild-type littermate. (E) ATXN2-luc detected in the OB and CB of a 7-month-old ATXN2-luc transgenic mouse and lack of signal in a wild-type littermate. (F–L) IF detection of endogenous Atxn2 in the OB of wild-type C57Bl/6 mice. (F) Ataxin-2 detected with ataxin-2 mAb in mitral cells and tufted cells in the glomerular layer (×10 magnification). (G) Same section as (F) stained with DAPI. (H) Merge of (F) and (G). (I) Ataxin-2 detected with polyclonal antibody 1080 in mitral cells and tufted cells in the glomerular layer (×20 magnification). (J) Same section as (I) stained with anti-GAD65/67. (K) Same section as (I) stained with DAPI. (L) Merge of (I)–(K). Note that no ataxin-2 was detected in GAD65/67-positive interneurons. Labels: T, tufted cells; Gr, granule layer; M, mitral layer; P, external plexiform layer; Gl, glomerular layer. Bars = 100 μm.