Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation
(0 available);
any
Gpm6b mutation
(5 available)
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nervous system
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• irregularities are detected at the axon-myelin interface including; irregular width of the extracellular periaxonal space and occasional apparent fusion of the adaxonal glial membrane with the axonal membrane
• occasionally the axon-myelin apposition is severely disturbed
• rarely invaginations of the oligodendroglial plasma membrane into the axoplasm are seen
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• double myelination is occasionally seen
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behavior/neurological
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N |
• no defects in motor function are detected in mice at up to 24 months of age
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation
(0 available);
any
Gpm6b mutation
(5 available)
Plp1tm1Kan mutation
(1 available);
any
Plp1 mutation
(19 available)
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mortality/aging
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• most die by 4 - 5 months of age with none living past 6 months of age
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behavior/neurological
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• unable to remain on a horizontal bar without support at all ages tested
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• hunchback at 2 - 3 months of age
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• fail to grasp objects with the forelimbs
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muscle
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• hindlimb spasticity is seen at 10 days of age
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• at 2 - 3 months of age mice always have spastic hindlimbs
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nervous system
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• apoptotic oligodendrocytes are seen in the CNS
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• pathology is more severe in the white matter than in the gray matter
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• thickness is reduced to 2/3 that of controls
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• thickness is reduced to 2/3 that of controls
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• reactive microgliosis accompanies neurodegeneration
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• accompanies neurodegeneration
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• processes are longer and thicker and are often seen engulfing axons with a noncompacted cellular process
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• absence of proteolipids, decrease in cholesterol content
• intraperiod lines are partly condensed
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• pathology is more severe in the white matter than in the gray matter
• degeneration is also seen in the retina and the PNS
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• axonal swellings are seen earlier and more frequently than in Plp single null mice
• axonal degeneration in the optic nerve at 4.5 months of age
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• at P14 the optic nerve lacks myelin and at 1 month of age myelination of the optic nerve and spinal cord are clearly delayed
• myelination in the striatal white matter and in spinal cord gray matter is 86% and 67% that of controls
• however, most large caliber axons are myelinated
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vision/eye
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• no reproducible responses are detected at 19 - 25 weeks of age
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hearing/vestibular/ear
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N |
• distortion product otoacoustic emissions are present and major degeneration of the VIIIth cranial nerve is not seen
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• waves II - V (reflecting CNS function) are significantly delayed in mice at 2.5 months of age
• the delay in waves II - V is less pronounced at 4.5 months of age
• at both 2.5 and 4.5 months of age wave III is most delayed
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• moderate pantonal hearing loss across all frequencies
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cellular
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• apoptotic oligodendrocytes are seen in the CNS
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• apoptotic cells are seen in the CNS, some of which are oligodendrocytes
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hematopoietic system
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• reactive microgliosis accompanies neurodegeneration
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immune system
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• reactive microgliosis accompanies neurodegeneration
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homeostasis/metabolism
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation
(0 available);
any
Gpm6b mutation
(5 available)
Plp1tm1Kan mutation
(1 available);
any
Plp1 mutation
(19 available)
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mortality/aging
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• die at 7 - 12 months of age later than males
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behavior/neurological
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• progressive motor impairments similar to males but milder at 4 - 6 weeks of age
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|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation
(0 available);
any
Gpm6b mutation
(5 available)
Plp1tm1Kan mutation
(1 available);
any
Plp1 mutation
(19 available)
|
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mortality/aging
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• die at 7 - 12 months of age later than males
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behavior/neurological
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• progressive motor impairments similar to males but milder at 4 - 6 weeks of age
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Allelic
Composition |
Gpm6btm1Kan/Y
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Genetic
Background |
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J |
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation
(0 available);
any
Gpm6b mutation
(5 available)
|
|
|
nervous system
|
|
• irregularities are detected at the axon-myelin interface including; irregular width of the extracellular periaxonal space and occasional apparent fusion of the adaxonal glial membrane with the axonal membrane
• occasionally the axon?myelin apposition is severely disturbed
• rarely invaginations of the oligodendroglial plasma membrane into the axoplasm are seen
|
|
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• double myelination is occasionally seen
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behavior/neurological
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N |
• no defects in motor function are detected in mice at up to 24 months of age
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Analysis Tools