The Fig4Floxed/Floxed, P0-Cre and Fig4Floxed/plt, P0-Cre mutants that we generated with conditional ablation of Fig4 in Schwann cells displayed demyelinating features such as myelin debris and degeneration, onion bulbs and decrease of NCV, thus recapitulating demyelination of the CMT4J neuropathy. Here, FIG4 is linked to neuropathy.