Figure 1.

Characterization of transgene integration site, and C9orf72 protein levels in C9-BAC mice. (A) BAC construct containing human C9orf72 gene and 51.6 kb and 19.4 kb of upstream (orange) and downstream (blue) flanking sequence, respectively. (B) Map showing breakpoint and integration of the BAC on mouse chromosome 6. (C) Map showing insertion of C9orf72 transgene in Vgll4 gene and qPCR showing expression of Vgll4 in NT and C9–500 mice. (D, E) Protein blots showing NT, C9–500 and Baloh-Jax mice probed with α-C9orf72 antibody. (F) Protein blot quantitation. Data information: Statistical analyses for panel F were performed using one-way ANOVA with Bonferroni correction for multiple comparisons with mean +/− SEM shown; not significant (ns) = P > 0.05.