Several muscle signaling pathways that regulate metabolism, inflammation and fibrosis have been described to be dysregulated in LAMA2-CMD patients and animal models, including ERK, NFκB and STAT3 (Carmignac et al., 2011a; de Oliveira et al., 2014; Durbeej, 2015; Elbaz et al., 2012, 2015; Nguyen et al., 2019; Nunes et al., 2017; Taniguchi et al., 2006; Mehuron et al., 2014). Here, NFKB1 is linked to congenital muscular dystrophy.