Mouse Genome Informatics
tg1
    Tg(Mpz)80.2Wra/0
involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• reduced life span

behavior/neurological
• weak grip strength
• wobbling gait

growth/size/body

muscle
• atrophy of the paraspinal and hindlimb musculature
• leg muscles exhibit angulated and atrophic fibers suggestive of denervation

nervous system
• 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

reproductive system