Several mouse models of NM have been developed either by knocking out (KO) an endogenous gene (e.g. Nebulin-KO, Klhl40-KO, Cfl2-KO) or overexpressing a mutant protein via transgenesis (Tg) or knock-in (KI) [e.g. Tg(ACTA1D286G), KI(ACTA1H40Y), Tg(TPM3M9R)] (Agrawal et al., 2012; Bang et al., 2006; Corbett et al., 2001; Garg et al., 2014; Ravenscroft et al., 2011a, ,b; Witt et al., 2006). The gene discussed is KLHL40; the disease is nemaline myopathy.