• reduced life span

• weak grip strength

• wobbling gait

• atrophy of the paraspinal and hindlimb musculature

• leg muscles exhibit angulated and atrophic fibers suggestive of denervation

• impairment of axon sorting by Schwann cells; this axon sorting defect is more evident at P5 and P14 than at P28

• sciatic nerve shows a mixture of fibers with thin myelin sheaths for the diameter of axon and fibers in which single Schwann cells ensheath axons, but form no myelin

• sciatic nerve development is arrested at the stage in which Schwann cells are segregating large axons away from bundles of mixed caliber axons

• severe dysmyelination resulting from a developmental delay, with rare evidence of active demyelination or axonal degeneration

• the motor branch (quadriceps) are more dysmyelinated than the sensory branch (saphenous) of femoral nerve by P10

• ventral roots are more dysmyelinated than dorsal roots

• P28 sciatic nerve exhibits hypomyelination and an increase in the number of Schwann cell nuclei

• nerve conduction velocities are reduced to about 2 m/s with temporal dispersion of the compound motor action potential, suggesting dysmyelinating neuropathy