Conclusion In summary, we generated ADAM22-deficient mice and proved that ADAM22 plays an essential role in both the CNS and the PNS. The results of this study suggest that ADAM22 is involved in human diseases such as epilepsy and peripheral neuropathy. The human ADAM22 gene is relatively large, at 300 kb in length, and is comprised of more than 30 exons. It is assigned on 7q21, where several chromosomal aberrations that are accompanied by neurological disorders have been identified [29,30]. Further detailed molecular function analysis of ADAM22 may lead to progress in uncovering the mechanisms that underlie certain human neurological diseases.