In this study, we generated a knock in mouse model carrying the naturally occurring NGF mutation, NGFR100W, to model HSAN V. This research is the first to directly investigate a mice model for NGFR100W and HSAN V. We found that the beta-NGFR100W knock-in homozygous mice had a pre-mature death within 2 months. This evidence concerns the gene NGF and hereditary sensory and autonomic neuropathy.