Myelin abnormality in Charcot–Marie–Tooth type 4J recapitulates features of acquired demyelination

Abstract

Myelin change in CMT4J recapitulates the features of acquired demyelinating neuropathies. This pathology is not Schwann cell autonomous. Instead, it relates to systemic processes involving interactions of multiple cell types and abnormally elevated intracellular Ca²⁺ . Injection of a Ca²⁺ chelator into Fig4^-/- mice improved segmental demyelination, thereby providing a therapeutic strategy against demyelination. Ann Neurol 2018;83:756-770.