Peripheral Neuropathy in the Adreno-myelo-neuropathy Mouse Model

Abstract

AbstractThe Abcd1 knockout mouse mimics human adreno-myelo-neuropathy (AMN), thus contributes to a better understanding of disease mechanisms, which yet remain poorly identified. Only limited information is available about peripheral neuropathy (PN), although a notable component of AMN pathology besides myelopathy. To enrich our knowledge of PN, the current study reports the clinical, electromyographic and morphological aspects of peripheral neuropathy. We found that despite obvious electron microscopy anomalies in sciatic nerve axons, nerve conduction was nearly normal and did not seem to contribute significantly to the impaired motor performances of the Abcd1-/- mouse.