Phenotypes Associated with This Genotype

Genotype
MGI:4887891

• Allelic Composition: Tg(Plp)66Kan/Tg(Plp)66Kan
• Genetic Background: B6NCrl.Cg-Tg(Plp)66Kan

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

mortality/aging

premature death ( J:156106 )

• mutants die prior to P60

behavior/neurological

tremors ( J:156106 )

• mutants exhibit tremor starting at 2 weeks of age, followed later by seizures

seizures ( J:156106 )

• mutants develop seizures following tremors

nervous system

seizures ( J:156106 )

• mutants develop seizures following tremors

abnormal oligodendrocyte apoptosis ( J:156106 )

• mutants exhibit an increase in the number of apoptotic oligodendrocytes by P20

abnormal oligodendrocyte morphology ( J:156106 )

• oligodendrocytes show swelling of the Golgi apparatus and numerous autophagic vacuoles by P20

abnormal myelin sheath morphology ( J:156106 )

• markedly thinner myelin sheath

demyelination ( J:156106 )

• mutants exhibit marked dymyelination by P20

cellular

abnormal oligodendrocyte apoptosis ( J:156106 )

• mutants exhibit an increase in the number of apoptotic oligodendrocytes by P20

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Pelizaeus-Merzbacher disease		DOID:3210	OMIM:312080	J:156106