Trembler 2 Jackson heterozygotes can be identified by approximately 3 weeks of age by a rapid tremor and unsteady, wobbly gait.  They have spasticity in the muscles of the lower back and limbs and reduced ability to organize the hind limb movements.  These phenotypes derive from peripheral demyelination.  Although heterozygotes for this allele have not been found to die prematurely, male fertility is reduced.  Some, but not all, heterozygotes have elevated auditory brainstem responses.  This allele causes a less severe phenotype than the trembler mutation, with no paralysis, seizures, or juvenile mortality. 

This comparatively mild point mutation in the second transmembrane domain provides an excellent model for Dejerine-Sottas syndrome, Roussy-Levy syndrome, recurrent neuropathy with pressure palsies, and types 1A and 1E Charcot-Marie-Tooth disease.

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C57BL/6J-Pmp22<Tr-2J>/GrsrJ Frozen Embryo