Mouse Genome Informatics
tg1
    Tg(Plp)66Kan/Tg(Plp)66Kan
B6NCrl.Cg-Tg(Plp)66Kan
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 IDRef(s)
Pelizaeus-Merzbacher Disease; PMD 312080 J:156106


Mouse Genome Informatics
tg2
    Tg(Plp)66Kan/Tg(Plp)66Kan
involves: C57BL * DBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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


Mouse Genome Informatics
tg3
    Tg(Plp)66Kan/0
B6NCrl.Cg-Tg(Plp)66Kan
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 IDRef(s)
Pelizaeus-Merzbacher Disease; PMD 312080 J:156106