Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Cnp-MAPT*P301L)6Vle mutation
(0 available)
Tg(Cnp-SNCA)M2Vle mutation
(1 available)
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behavior/neurological
nervous system
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• mice exhibit accumulation of tau and alpha-synuclein amyloidogenic inclusions in oligodendrocytes as early as 12 months of age
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• mice exhibit accumulation of tau and alpha-synuclein amyloidogenic inclusions in oligodendrocytes as early as 12 months of age
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Cnp-SNCA)M2Vle mutation
(1 available)
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behavior/neurological
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• mice show a decrease in endurance time on the rotarod treadmill test beginning at 7-9 months of age
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• mice show a progressive reduction in grip strength on a wire hanging test with advancing age
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nervous system
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• 8% and 16% reduction in brain weight at 9 and 24 months of age, respectively
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• reduction in the white matter in 24 month old mice
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• progressive brain atrophy, with mice showing an 8% and 16% reduction in brain weight at 9 and 24 months of age, respectively
• brain atrophy reflects a reduction in the neuropil and white matter
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• decrease in the number of oligodendroctyes at 24 months of age, indicating oligodendrocytic degeneration
• oligodendrocytes contain degraded myelin, extensive accumulations of lysosomes, fragmented myelin in the cytoplasm, suggesting autophagocytosis of degenerated myelin
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• in the brain and spinal cord
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• degenerated myelin sheaths associated with degenerating axons in the CNS, most prominently in the spinal cord
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• decrease in the number of neurons at 24 months of age, with neuronal loss most prominent in the lateral and medial columns of the spinal cord
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• secondary neuronal degeneration as a consequence of oligodendrocytic cytoplasmic inclusions
• loss of neurons in the lateral and medial columns of the spinal cord
• loss of myelinated axons and sprouting of unmyelinated axons of spinal ventral roots, indicating neuronal degeneration in the anterior horn of spinal cord
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• neuronal degeneration in the anterior horn of spinal cord
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• mice exhibit alpha-synuclein fibrillar aggregates resembling glial cytoplasmic inclusions in oligodendroctyes and the neuropil
• accumulation of filamentous human alpha-synuclein aggregates in oligodendroctyes and in neuropil with increasing age
• endogenous mouse alpha-synuclein aggregates in normal and degenerating axons and axon terminals in association with oligodendroglia with a tubular ultrastructure
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• neuronal and axonal degeneration as indicated by a redistribution of highly phosphorylated high-molecular weight neurofilament subunit proteins from axons into neuronal perikarya, detection of axonal spheroids with RMO24 in the cortex, gliosis in the brain and spinal cord, and Gap43 immunoreactivity surrounding neuronal perikarya
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• demyelination of oligodendroctyes
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mortality/aging
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N |
• life span is not affected
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Cnp-SNCA)M2Vle mutation
(1 available)
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nervous system
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N |
• mice do not develop tau or alpha-synuclein inclusions at 18 months of age
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Analysis Tools