Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice

► In this study we characterized the SMN2 transgenic mouse, Tg(SMN2)89Ahmb. It is the most commonly used SMN2 transgene in mouse models of spinal muscular atrophy. ► Tg(SMN2)89Ahmb integrated within the metabatropic glutamate receptor 7 (mGluR7)gene and created a hypomorphic allele. ► On a hybrid FVB/N background (70% FVB/N) this causes increased seizure susceptibility and a heightened fear response.