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Phenotypes Associated with This Genotype
Genotype
MGI:6191699
Allelic
Composition
Mpztm3.1Wra/Mpz+
Genetic
Background
FVB.129S2(Cg)-Mpztm3.1Wra
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mpztm3.1Wra mutation (0 available); any Mpz mutation (21 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice perform worse than wild-type mice on the rotating rod

cellular
• Schwann cell proliferation is increased in sciatic nerves
• however, no differences in Schwann cell apoptosis are seen

nervous system
• Schwann cell proliferation is increased in sciatic nerves
• however, no differences in Schwann cell apoptosis are seen
• Schwann cell development is delayed
• about 1% increase in sciatic nerve myelin period
• however, optic nerve myelin period is normal
• sciatic nerve segments have a coherence length of myelin reduced by about 30% and a period distortion that is about 25% greater, indicating more membrane packing irregularity
• sciatic nerves show thinner myelin and an increased average G-ratio of 0.77 compared to 0.67 in wild-type controls at 6 weeks of age
• small diameter axons are less affected than large diameter axons
• the number of myelinated axons is reduced in sciatic nerves
• evoked compound muscle action potential amplitudes are reduced but to a lesser extent than in homozygotes
• mice have slowed nerve conduction velocities at 6-8 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Charcot-Marie-Tooth disease type 1B DOID:0110152 OMIM:118200
J:241742


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
02/16/2021
MGI 6.16
The Jackson Laboratory