A progressive severe dysmyelinating neuropathy is in fact associated to MDC1A in humans and mouse models [17, 18, 49, 50], and it is already known that genetic repletion of laminin chain α2 in the skeletal muscles of mouse mutants does not completely revert the clinical phenotype due to the progression of the peripheral neuropathy [56]. Here, LAMA2 is linked to peripheral neuropathy.