Phenotypes associated with this allele

• Allele Symbol: Tg(PMP22)C22Fbas
• Allele Name: transgene insertion C22, Frank Baas
• Allele ID: MGI:5817407
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(PMP22)C22Fbas/?	B6.Cg-Tg(PMP22)C22Fbas	MGI:5824745

Genotype
MGI:5824745

tg1

• Allelic Composition: Tg(PMP22)C22Fbas/?
• Genetic Background: B6.Cg-Tg(PMP22)C22Fbas

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

nervous system

abnormal submucous nerve plexus morphology ( J:237901 )

• abnormal Remak fibers with an increase in membranous structures

abnormal Schwann cell morphology ( J:237901 )

• attenuated Schwann cell processes are observed

abnormal axon morphology ( J:237901 )

• abnormally large numbers of small axons are observed in some animals

• mean axon diameters in the motor femoral nerve at 3, 24, and 48 weeks are smaller that wild-type

axon degeneration ( J:237901 )

• atrophic fibers are observed as well as sequestration of axon organelles by Schwann cells

abnormal myelination ( J:237901 )

• myelination is delayed and never reaches control levels

• the phenotype is less severe than in Tg(PMP22)C3Fbas mice

demyelination ( J:237901 )

• demyelinated axons and myelin debris are sometimes found in associated Schwann cells

• small onion bulb formations, an indication of repeated demyelination and remyelination, are sometimes observed

dysmyelination ( J:237901 )

• peroneal nerve fibers from 3 week old mice are thinly myelinated or amyelinated as compared to wild-type

• the phenotype is less severe in Tg(PMP22)C3Fbas mice

• nerve fibers from motor branch of the femoral nerve have a lower number of myelinated fibers than wild-type or Tg(PMP22)C3Fbas mice

• partial or completely uncompacted myelin sheaths are present in peripheral nerves but not roots

• lumbar ventral roots have a higher proportion of abnormal fibers than dorsal roots or peripheral nerves

• 40% of peroneal and femoral nerve fibers are normally myelinated at 3 weeks increasing to 64% in adults, but never reaching 100%

hypermyelination ( J:237901 )

• many small diameter fibers are hypermyelinated

abnormal action potential ( J:237901 )

• mean compound muscle action potential (CMAP) amplitude is decreased at all ages as compared to wild-type and Tg(PMP22)C3Fbas mice

• mean compound nerve action potential (CNAP) amplitude is decreased as compared to wild-type and Tg(PMP22)C3Fbas mice

decreased nerve conduction velocity ( J:237901 )

• mean nerve conduction velocity is decreased as compared to Tg(PMP22)C3Fbas and wild-type mice

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:237901 )

• neuromuscular SHIRPA scores are increased from week 3 to 24 indicating a decrease in performance, particularly in tremor, gait, tail elevation, touch escape, grip strength and wire maneuver

• scores are higher than both wild-type and Tg(PMP22)C3Fbas mice

decreased locomotor activity ( J:237901 )

• reduced locomotor activity as compared to wild-type