Mice ablated for PrP (first-generation PrP knockout mice and prnp-ablated mice generated through genome editing) (Bremer et al., 2010; Nuvolone et al., 2016) and goats lacking PrP (Skedsmo et al., 2020) develop a progressive peripheral demyelinating neuropathy, whose mechanism is now foreseen. Here, PRNP is linked to demyelinating polyneuropathy.