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Phenotypes Associated with This Genotype
involves: C57BL/6J * CBA/Ca
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(PMP22)C22Clh mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• 6 male mice die suddenly between 2 and 5 months of age

nervous system
• Ramak fibers (bundles of unmyelinated axons surrounded by a single Schwann cell) show an excess of Schwann cell membrane formation
• Schwann cell g ratio is increased, indicating thin myelin sheaths
• decrease in the proportion of large myelinated fibers and an increase in the proportion of smaller ones
• increase in myelin periodicity of nerves
• uncompacted myelin is seen
• 42.4% of fibers are dysmyelinated

reproductive system
• male mice rarely mate

• at 3 weeks of age, mice shake when held in the air unlike wild-type mice
• at 3 weeks of age, mice react more than normal mice to sudden noises and other disturbances
• at 3 weeks of age, mice are unsteady when walking unlike wild-type mice
• mice progressively lose control of their hind legs

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Charcot-Marie-Tooth disease type 1A DOID:0110148 OMIM:118220
J:76795 , J:78221

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.11
The Jackson Laboratory