mortality/aging
|
• mutants die prior to P60
|
behavior/neurological
|
• mutants exhibit tremor starting at 2 weeks of age, followed later by seizures
|
nervous system
|
• 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 | |
