Fig. 1

Characterisation of Sbno2^−/− and GFAP-IL6 × Sbno2^−/− mice. A Exons 8 to 10 of Sbno2 were flanked with loxP sites (dotted line) and were excised by Cre recombinase. PCR primers (arrows) generated a 108 bp product from the WT locus (PCR Product A) or a 355 bp product from the recombined locus (PCR Product B). A probe against exons 9 to 11 (RNA transcript probe) was used to detect Sbno2 transcript by RPA. B PCR using tail DNA indicated that the unrecombined Sbno2 locus (PCR product A) was present in WT mice whereas the recombined locus (PCR product B) was present in Sbno2^−/− mice. NTC, no template control; POS, positive control. C RNA extracted from the cerebellum was used in RPA to measure Sbno2 transcript. Transcript was detectable in WT mice and was increased in GFAP-IL6 mice, but was not detectable in Sbno2^−/− or GFAP-IL6 × Sbno2^−/− mice (n = 3 per genotype). D Male or female mice were weighed at 1, 3 and 7 months of age (n = 6–15 per genotype). E Clinical scoring of gait in GFAP-IL6 and GFAP-IL6 × Sbno2^−/− measured development of ataxia (n = 7–18 per genotype). Weights at 30 weeks of age were compared by Mann–Whitney U test and presented as the mean ± SEM. The mean age of onset was 23 weeks for GFAP-IL6 mice and 18 weeks for GFAP-IL6 × Sbno2^−/− mice, although this was not significantly different. F All mice appeared physically normal at all ages examined. Representative images of 3-month-old male mice shown. G IL-6 could not be detected by ELISA in lysates of cerebellum from 1- or 3-month-old WT mice but was present in GFAP-IL6 and GFAP-IL6 × Sbno2^−/− mice (n = 3 per genotype). *, p < 0.05 compared with WT; ^, p < 0.05 compared with Sbno2^−/−; #, p < 0.05 compared with GFAP-IL6