Mouse Genome Informatics
ot
    Plp1tm1Kan/Y
involves: 129S1/Sv * 129X1/SvJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• increased numbers of microglia accompany degenerative changes (J:48031)
• mild astrocytosis accompanies degenerative changes (J:48031)
• occasionally inner tongue processes of pligdendrocytes contain degenerated organelles (J:48031)
• primary glial cultures have increased numbers of oligodendrocytes starting at the second division and persisting through at least the fourteenth division; however no increase in oligodendrocyte proliferation is seen (J:106182)
• in primary glial cultures oligodendrocyte sheets appear larger, vacuolated, and broken suggesting decreased stability of the myelin sheet (J:106182)
• at 6 - 8 weeks, focal axonal swellings containing organelles are seen mostly in areas where small diameter axons predominate throughout the white and gray matter (J:48031)
• by 1 year, numerous large axonal swellings are seen in the optic nerve and spinal cord with some also seen in Purkinje cell axons (J:48031)
• after 1 year, axonal degeneration is seen in the optic nerve and fasciculus gracilis; however, no signs of peripheral neuropathy are seen (J:48031)
• in areas of axonal swelling the myelin sheath becomes attenuated and is eventually lost though slippage (J:48031)
• decrease in cholesterol content (J:193310)
• evidence of demyelination is seen at 22 months of age (J:48031)

behavior/neurological
• at 16 months of age impaired performance in a rotarod test is seen (J:48031)
• at 16 months of age impaired performance in a rotarod test is seen (J:48031)
• older mice develop a slow gait (J:48031)

hematopoietic system
• increased numbers of microglia accompany degenerative changes (J:48031)

immune system
• increased numbers of microglia accompany degenerative changes (J:48031)

homeostasis/metabolism
• in myelin (J:193310)

Mouse Models of Human Disease
OMIM IDRef(s)
Spastic Paraplegia 2, X-Linked; SPG2 312920 J:48031