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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(PMP22)C22Clh
transgene insertion C22, Clare Huxley
MGI:2183770
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(PMP22)C22Clh/0 involves: C57BL/6J * CBA/Ca MGI:3845794


Genotype
MGI:3845794
tg1
Allelic
Composition
Tg(PMP22)C22Clh/0
Genetic
Background
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
mortality/aging
• 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

behavior/neurological
• 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
OMIM ID Ref(s)
Charcot-Marie-Tooth Disease, Demyelinating, Type 1A; CMT1A 118220 J:76795 , J:78221





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory