Mouse models for each of the three Shank proteins have been created to better understand the role of these proteins in vivo and their contributions to ASDs. The first model was created by deleting exons 14–15 of Shank1 (Hung et al., 2008) where homozygous deletion (Shank1−/−) results in some ASD-like features (Silverman et al., 2011; Wöhr et al., 2011; Sungur et al, 2014). Two different lines of Shank2 mutant mice have been reported, which involved deletion of exon 7 (Schmeisser et al., 2012) or exons 6–7 (Won et al., 2012) of the Shank2a isoform, corresponding to exon 17 and exons 16–17, respectively, of full length Shank2 (Jiang and Ehlers, 2013). Both lines are assumed to have disrupted function of all Shank2 isoforms and display stereotypy, diminished social interactions, and impaired vocalizations (Schmeisser et al., 2012; Won et al., 2012; Ey et al., 2013).
