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Phenotypes Associated with This Genotype
Genotype
MGI:6276667
Allelic
Composition
Tg(Mpz)88.1Mfel/0
Genetic
Background
FVB/N-Tg(Mpz)88.1Mfel
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
behavior/neurological

muscle
• muscular atrophy is evident
• mice exhibit variable signs of muscle weakness such as clenching of the paws, dragging of hind limbs and floppy tail

nervous system
• sciatic nerves shows widening of myelin lamellae
• poorly compacted areas show absence of the intraperiod line and occasionally, the major dense line is uncompacted, indicating packing abnormalities
• occasional sciatic nerve fibers show thick and redundant myelin at P28, likely indicating myelin outfolding or tomacula
• more frequent tomacula are seen at P7
• tomacula appear to originate from the paranodal region and contain poorly compacted myelin
• occasionally older mice show signs of axonal degeneration
• occasionally older mice exhibit demyelinating figures and short internodes suggesting remyelination
• however, no onion bulbs are seen at any age
• sciatic nerves at P28 show hypomyelination, with thin or absent myelin sheaths on many large caliber axons
• sciatic nerves at 7 months show more pronounced hypomyelination, with most large caliber axons showing very thin or absent myelin

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Charcot-Marie-Tooth disease type 1B DOID:0110152 OMIM:118200
J:77658


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
02/23/2021
MGI 6.16
The Jackson Laboratory