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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Mpz)88.4Mfel
transgene insertion 88.4, M Laura Feltri
MGI:2384594
Summary 3 genotypes


Genotype
MGI:6277901
cx1
Allelic
Composition
Mpztm1Msch/Mpz+
Tg(Mpz)88.4Mfel/0
Genetic
Background
FVB.Cg-Mpztm1Msch Tg(Mpz)88.4Mfel
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mpztm1Msch mutation (0 available); any Mpz mutation (28 available)
Tg(Mpz)88.4Mfel mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• myelin sheaths are thinner
• myelin lamellae are widened
• however, mice show improved hypomyelination
• mice show tomacula that are not improved




Genotype
MGI:6277904
cx2
Allelic
Composition
Mpztm1Msch/Mpztm1Msch
Tg(Mpz)88.4Mfel/0
Genetic
Background
FVB.Cg-Mpztm1Msch Tg(Mpz)88.4Mfel
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mpztm1Msch mutation (0 available); any Mpz mutation (28 available)
Tg(Mpz)88.4Mfel mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• abnormal compaction is not improved
• two types of abnormally compacted fibers are seen: rare fibers in which both the intraperiod and major dense line are absent and majority of fibers with myelin that appears compacted but has abnormal periodicity due to a wide (but not absent) intraperiod line
• myelin sheaths are thinner
• tomacular are readily seen at P28
• tomacula are not improved and are worsened
• hypomyelination is only slightly improved




Genotype
MGI:6276671
tg3
Allelic
Composition
Tg(Mpz)88.4Mfel/0
Genetic
Background
FVB/N-Tg(Mpz)88.4Mfel
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
• mice exhibit variable signs of muscle weakness such as clenching of the apws, dragging of hind limbs and floppy tail
• symptoms are least severe in this line

nervous system
• sciatic nerves show uncompaction of the myelin sheath
• sciatic nerves show tomacula
• sciatic nerves show hypomyelination

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





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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
12/10/2024
MGI 6.24
The Jackson Laboratory