mortality/aging
• almost all die at around 30 days of age
|
nervous system
• at 24 days of age, exhibit increased Rosenthal fiber formation and higher levels of insoluble Gfap than either single mutant
• astrocytes are hypertrophied in the regions of brain that normally show high levels of Gfap expression
|
behavior/neurological
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Alexander disease | DOID:4252 |
OMIM:203450 |
J:114689 |