Tuning PAK Activity to Rescue Abnormal Myelin Permeability in HNPP.

ABSTRACT: Schwann cells in the peripheral nervous systems extend their membranes to wrap axons concentrically and form the insulating sheath, called myelin. The spaces between layers of myelin are sealed by myelin junctions. This tight insulation enables rapid conduction of electric impulses (action potentials) through axons. Demyelination (stripping off the insulating sheath) has been widely regarded as one of the most important mechanisms altering the action potential propagation in many neurological diseases. However, the effective nerve conduction is also thought to require a proper myelin seal through myelin junctions such as tight junctions and adherens junctions. In the present study, we have demonstrated the disruption of myelin junctions in a mouse model (Pmp22+/-) of hereditary neuropathy

SUBMITTER: Hu B 

PROVIDER: S-EPMC5008806 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature