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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Plp)66Kan
transgene insertion 66, Klaus-Armin Nave
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
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die prematurely at 4-6 months of age

behavior/neurological
• severe seizures and convulsions

nervous system
• severe seizures and convulsions
• astrocytosis of the spinal cord
• dysmyelination of the spinal cord




Genotype
MGI:4887891
tg2
Allelic
Composition
Tg(Plp)66Kan/Tg(Plp)66Kan
Genetic
Background
B6NCrl.Cg-Tg(Plp)66Kan
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants die prior to P60

behavior/neurological
• mutants exhibit tremor starting at 2 weeks of age, followed later by seizures
• mutants develop seizures following tremors

nervous system
• mutants develop seizures following tremors
• mutants exhibit an increase in the number of apoptotic oligodendrocytes by P20
• oligodendrocytes show swelling of the Golgi apparatus and numerous autophagic vacuoles by P20
• markedly thinner myelin sheath
• mutants exhibit marked dymyelination by P20

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

Mouse Models of Human Disease
OMIM ID Ref(s)
Pelizaeus-Merzbacher Disease; PMD 312080 J:156106




Genotype
MGI:2388045
tg3
Allelic
Composition
Tg(Plp)66Kan/Tg(Plp)66Kan
Genetic
Background
involves: C57BL * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die at approximately 2 months of age

behavior/neurological
• apparent by 2 weeks of age and progressive severity with age
• apparent by 2 weeks of age and progressive severity with age
• apparent by 2 weeks of age and progressive severity with age

nervous system
• apparent by 2 weeks of age and progressive severity with age
• widespresd astrocytosis in white matter areas of hypomylelinated structures
• increased number of processes
• 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
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• at 15-18 months of age, mutants develop ataxia

growth/size/body
• at 15-18 months of age, mutants begin to lose weight

nervous system
• slightly thinner myelin sheath
• older mice exhibit axonal degeneration preferentially affecting smaller diameter fibers
• mice show demyelination starting from 15-18 months of age

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

Mouse Models of Human Disease
OMIM ID Ref(s)
Pelizaeus-Merzbacher Disease; PMD 312080 J:156106





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last database update
07/19/2016
MGI 6.04
The Jackson Laboratory