Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1jp mutation
(2 available);
any
Plp1 mutation
(19 available)
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1jp mutation
(2 available);
any
Plp1 mutation
(19 available)
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mortality/aging
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• most hemizygotes die between 25 and 30 days of age with some dying as early as 20 days of age and some as late as 34 days of age
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nervous system
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• only 1.2% of axons in optic nerve are myelinated at 19 to 21 days of age showing that even on the B6C3 background there is reduced myelination compared with mice hemizygous for the myelin synthesis deficiency (msd) allele on this same background, in which 3.6% of optic nerve axons are myelinated
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behavior/neurological
growth/size/body
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1jp mutation
(2 available);
any
Plp1 mutation
(19 available)
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behavior/neurological
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• onset is developmentally delayed
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• up to P8, mice exhibit less ultrasonic vocalizations compared with wild-type mice
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nervous system
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• as myelination advances oligodendrocytes become abnormal with lipid inclusions and multimembrane tubes unlike in wild-type mice
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• during the early period of myelination (1 to 6 days of age)
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• cell cycle length of astrocytes is increased by 5 to 6 hours compared with wild-type cells
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(J:5611)
• microsomes prepared from brains of 18 day old homozygotes show a reduction in fatty acid chain elongation activity with arachidoyl CoA, behenoyl CoA, and palmitoyl CoA substrates
(J:160743)
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growth/size/body
vision/eye
Allelic
Composition |
Plp1jp/Y
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Genetic
Background |
Not Specified |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1jp mutation
(2 available);
any
Plp1 mutation
(19 available)
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|
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mortality/aging
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• mice die at around 28 days with some living as long as 40 days
(J:288)
• death usually occurs by 30 days commonly after a seizure
(J:13141)
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nervous system
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• when disturbed or handled mice exhibit convulsions characterized by body tension with head thrown back, fore limbs scrabbling and hind limbs stretched sideways for about 5 seconds sometimes followed by rigid extensor tetanus lasting 20 seconds unlike wild-type mice
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• generalized seizures without focal onset occur by 4 weeks of age
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• brain contains traces of cholesterol ester
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• cytoplasmic nonpolar lipids are observed in the basis pedunculi, optic tract and white matter of the cerebellum and spinal cord; these cells appear to be fatty macrophages and are first observed in the 12 day old brain
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• freeze-etching of optic nerves shows that plasmalemma of astrocytes have twice normal numbers of assemblies of 7nm particles, which are similar to type III gap junctions, and these appear to unite abnormally with type I junctions
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• freeze-etching of optic nerves shows that the oligodendroglia plasma membrane is highly convoluted with numerous irregular invaginations and elevations
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• freeze-etching of optic nerves shows abnormally smooth protoplasmic fracture faces and exoplasmic fracture faces and there are fewer than normal intramyelinic tight junctions
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• little myelin is found in any region of the central nervous system
• myelination is observed in the peripheral nervous system
• general architecture of gray and white matter is normal
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behavior/neurological
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• mice exhibit intention tremors
(J:288)
• tremors are first observed at about10-12 days of age
(J:13141)
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• some animals exhibit hindlimb weakness by weaning age
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• when disturbed or handled mice exhibit convulsions characterized by body tension with head thrown back, fore limbs scrabbling and hind limbs stretched sideways for about 5 seconds sometimes followed by rigid extensor tetanus lasting 20 seconds unlike wild-type mice
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• generalized seizures without focal onset occur by 4 weeks of age
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Analysis Tools