Mouse Genome Informatics
tg
    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