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Phenotypes Associated with This Genotype
Genotype
MGI:3620242
Allelic
Composition
Plp1tm1Kan/Y
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• increased numbers of microglia accompany degenerative changes
• mild astrocytosis accompanies degenerative changes
• 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
• by 1 year, numerous large axonal swellings are seen in the optic nerve and spinal cord with some also seen in Purkinje cell axons
• after 1 year, axonal degeneration is seen in the optic nerve and fasciculus gracilis; however, no signs of peripheral neuropathy are seen
• 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

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

hematopoietic system
• increased numbers of microglia accompany degenerative changes

immune system
• increased numbers of microglia accompany degenerative changes

homeostasis/metabolism

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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/22/2016
MGI 6.06
The Jackson Laboratory