Disruption of lysosomal homeostatis and extracellular cathepsin release by Schwann cells in Charcot-Marie-tooth disease type 1A

Abstract

Conclusions: We have identified a novel promyelinating factor, PAK2, that acts as a critical convergence point for multiple promyelinating signaling pathways. The promyelination by PAK2 is Schwann cell-autonomous. Myelin lipids, identified as inhibitors or activators of PAK2, may be utilized to develop therapies for repairing abnormal myelin in peripheral neuropathies.