Mouse Genome Informatics
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• swollen axons are seen
• some attenuated myelin sheaths are seen around swollen axons
• however, in non swollen regions sheaths are of normal thickness
• at 8 months of age occasional degenerating fibers are seen in the cervical region of the fasciculus gracilis
• at 18 months of age pronounced axonal degeneration is seen in the cervical segments in the thoracic region but not in the lumbar dorsal columns

• gait ataxia is seen by 18 months of age
• weakness particularly of the hind limbs develops by 18 months of age

Mouse Models of Human Disease
Pelizaeus-Merzbacher Disease; PMD 312080 J:146665