KLHL9 (Cirak et al., 2010), KBTBD13 (Sambuughin et al., 2010), KLHL40 (Ravenscroft et al., 2013), and KLHL41 (Gupta et al., 2013) have been implicated in distal and nemaline myopathy. Here, KLHL41 is linked to nemaline myopathy.