Phenotypes associated with this allele

• Allele Symbol: Tg(Plp)66Kan
• Allele Name: transgene insertion 66, Klaus-Armin Nave
• Allele ID: MGI:2388028
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Tg(Plp)66Kan/0 Tg(Plp)72Kan/0	involves: C57BL/6 * DBA	MGI:5776482
tg2	Tg(Plp)66Kan/Tg(Plp)66Kan	B6NCrl.Cg-Tg(Plp)66Kan	MGI:4887891
tg3	Tg(Plp)66Kan/Tg(Plp)66Kan	involves: C57BL * DBA	MGI:2388045
tg4	Tg(Plp)66Kan/0	B6NCrl.Cg-Tg(Plp)66Kan	MGI:4887901

Genotype
MGI:5776482

cx1

• Allelic Composition: Tg(Plp)66Kan/0; Tg(Plp)72Kan/0
• Genetic Background: involves: C57BL/6 * DBA

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

mortality/aging

premature death ( J:17373 )

• mice die prematurely at 4-6 months of age

behavior/neurological

convulsive seizures ( J:17373 )

• severe seizures and convulsions

nervous system

convulsive seizures ( J:17373 )

• severe seizures and convulsions

astrocytosis ( J:17373 )

• astrocytosis of the spinal cord

dysmyelination ( J:17373 )

• dysmyelination of the spinal cord

Genotype
MGI:4887891

tg2

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

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

Genotype
MGI:2388045

tg3

• Allelic Composition: Tg(Plp)66Kan/Tg(Plp)66Kan
• Genetic Background: involves: C57BL * DBA

mortality/aging

premature death ( J:17373 )

• die at approximately 2 months of age

behavior/neurological

tremors ( J:17373 )

• apparent by 2 weeks of age and progressive severity with age

ataxia ( J:17373 )

• apparent by 2 weeks of age and progressive severity with age

seizures ( J:17373 )

• apparent by 2 weeks of age and progressive severity with age

nervous system

seizures ( J:17373 )

• apparent by 2 weeks of age and progressive severity with age

astrocytosis ( J:17373 )

• widespresd astrocytosis in white matter areas of hypomylelinated structures

• increased number of processes

demyelination ( J:17373 )

• progressive with age

• more pronounced in forebrain and optic nerves

• less severe in the brainstem and spinal cord

• within the spinal cord, hypomyelination was more severe in the dorsal columns than in the ventral columns

Genotype
MGI:4887901

tg4

• Allelic Composition: Tg(Plp)66Kan/0
• Genetic Background: B6NCrl.Cg-Tg(Plp)66Kan

behavior/neurological

ataxia ( J:156106 )

• at 15-18 months of age, mutants develop ataxia

growth/size/body

weight loss ( J:156106 )

• at 15-18 months of age, mutants begin to lose weight

nervous system

abnormal myelin sheath morphology ( J:156106 )

• slightly thinner myelin sheath

axon degeneration ( J:156106 )

• older mice exhibit axonal degeneration preferentially affecting smaller diameter fibers

demyelination ( J:156106 )

• mice show demyelination starting from 15-18 months of age

skeleton

kyphosis ( J:156106 )

• at 15-18 months of age, mutants develop kyphosis

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