Biallelic homozygous MPV17 variants have been reported to result in juvenile-onset isolated peripheral sensorimotor neuropathy (CMT2EE) without hepatocerebral involvement, as occurs in autosomal recessive (AR) mitochondrial DNA depletion syndrome 6 (Navajo neurohepatopathy). This evidence concerns the gene MPV17 and mitochondrial DNA depletion syndrome 6 (hepatocerebral type).