Mouse Genome Informatics
ot
    Plp1tm1Kan/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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

behavior/neurological
• 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
OMIM IDRef(s)
Pelizaeus-Merzbacher Disease; PMD 312080 J:146665