Phenotypes Associated with This Genotype

Genotype
MGI:3828308

• Allelic Composition: Plp1^tm1Frca/Y
• Genetic Background: B6.129-Plp1^tm1Frca

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

nervous system

abnormal myelin sheath morphology ( J:143305 )

♂ • at 1 month, optic nerves exhibit rare myelin whorls and some axons with loose myelin wraps unlike in wild-type mice

♂ • at 6 months, mice exhibit more pronounced myelin whorls than at 3 months

axon degeneration ( J:143305 )

♂ • at 2 months, axonal degeneration is occasionally observed but the frequency of occurrence does not increase with age

demyelination ( J:143305 )

♂ • at 3 months, mice exhibit myelin membrane degeneration unlike in wild-type mice

♂ • at 6 months, mice exhibit more pronounced myelin degeneration than at 3 months

behavior/neurological

behavior/neurological phenotype ( J:143305 )

♂N • despite impaired coordination, mice exhibit normal grip strength and exploratory behavior

impaired coordination ( J:143305 )

♂ • at 3 and 6 months, the latency to fall off a rotarod is decreased compared to in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Pelizaeus-Merzbacher disease		DOID:3210	OMIM:312080	J:143305