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Phenotypes Associated with This Genotype
Genotype
MGI:5588113
Allelic
Composition
Egr2tm2(cre)Pch/Egr2+
Gt(ROSA)26Sortm8(Map2k1*,-EGFP)Rsky/?
Genetic
Background
involves: 129S2/SvPas * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation (2 available); any Egr2 mutation (32 available)
Gt(ROSA)26Sortm8(Map2k1*,-EGFP)Rsky mutation (1 available); any Gt(ROSA)26Sor mutation (1062 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• onion bulb structures are seen indicating myelin degeneration and regeneration
• however, stacking of myelin sheaths in compact myelin is unaffected
• noncompact myelin structures are more abundant
• myelin sheaths are thicker at P15 and P30 and myelin continues to grow radially such that the mean myelin area in peripheral nerves at P90 is increased 6-fold
• increase in myelin thickness is particularly pronounced in small-diameter fibers
• aberrant paranodal myelin (tomaculi) and infolded myelin loops
• increase in the diameter of sciatic nerves due to massive hypermyelination and reduced axonal packing
• myelination begins prematurely and mice show continuous myelin growth
• increase in the number of myelinated fibers per field at P0
• myelin abnormalities are seen at P30-P90 but not at P15, indicating that they result from massive hypermylination
• treatment with the mTORC1 inhibitor everolimus dampens myelin growth in the nerves of mutants and completely rescues the decreased axonal packing
• ratio of compound action potentials in myelinated A and nonmyelinated C fibers (A/C fiber waves) is reduced and there is a reduction in numbers of active mechanoreceptive units after P60 indicating that many myelinated axons are not conducting
• D-hair mechanoreceptors show increased conduction velocities at P30, however no differences were seen after P60
• however, conduction velocities of other mechanosensory fibers are unchanged


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
01/06/2026
MGI 6.24
The Jackson Laboratory