Mouse Genome Informatics
ht
    Pmp22Tr-J/Pmp22+
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• Schwann cell nuclei numbers are increased (J:39953)
• Shwann cell numbers and rates of apoptosis are increased compared to in wild-type mice (J:134811)
• however, treatment with curcumin decreases apoptosis rates of Schwann cells (J:134811)
• treatment with curcumin increases axonal size
• mice exhibit minor structural changes in dorsal root ganglion cells
• 40% to 60% of axons lack myelination (J:39953)

behavior/neurological
• mice are unable to remain on a rotarod as long as wild-type mice
• however, treatment with curcumin improves coordination
• beginning at 4 to 6 weeks of age, mice exhibit abnormal gait with hindlimb spaying

muscle
• mice exhibit muscle wasting

Mouse Models of Human Disease
OMIM IDRef(s)
Charcot-Marie-Tooth Disease, Demyelinating, Type 1A; CMT1A 118220 J:3394 , J:98231