ADAM17 and Charcot-Marie-Tooth disease: CMT4B1 is a severe autosomal recessive neuropathy characterized by excessive aberrant myelin in the nerve.6 This morphology is similar to that observed in nerves of other CMT forms such as CMT4B2, B3, CMT4H and HNPP, the hereditary liability to pressure palsies.5 We recently explored efficacy of the niacin-mediated TACE activation as a strategy to decrease NRG1 type III signalling and ameliorate CMT neuropathies associated with excessive redundant myelin in the nerve.