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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Mpz)80.2Wra
transgene insertion 80.2, Lawrence Wrabetz
MGI:2447694
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Mpz)80.2Wra/0 involves: FVB/N MGI:2653547


Genotype
MGI:2653547
tg1
Allelic
Composition
Tg(Mpz)80.2Wra/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median life span is 4 months

homeostasis/metabolism
• collagen is increased within the perineurial compartment

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
• nerve development is arrested at the stage in which Schwann cells are segregating large axons away from bundles of mixed caliber axons
• Schwann cells incompletely retract their processes after segregation of large axons
• axon-sorting defect is more evident at P5 and P14 than at P28 and even at P45 and P90, some bundles of unsorted larger diameter axons remain indicating delayed axonal sorting
• 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
• mice exhibit severe dysmyelination that results from developmental delay and not myelin destruction
• sciatic nerves at P5 and P14 contain virtually no myelin while occasional patches of myelination fibers are seen at P28 and more frequently at P42
• sciatic nerves show a mixture of fibers with thin myelin sheaths and fibers in which single Schwann cells ensheath axons but form no myelin
• by P10, less than 1% of axons in the motor branch (quadriceps) of developing femoral nerve in 1:1 relationships with Schwann cells are myelinated compared to 100% in wild-type
• the motor branch (quadriceps) is more dysmyelinated than the sensory branch (saphenous) of femoral nerve by P10
• ventral roots are more dysmyelinated than dorsal root
• nerve conduction velocities are reduced to about 2 m/s with temporal dispersion of the compound motor action potential, suggesting dysmyelinating neuropathy

reproductive system

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
neuropathy DOID:870 J:78758





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last database update
10/29/2024
MGI 6.24
The Jackson Laboratory