Phenotypes associated with this allele

• Allele Symbol: Tg(Cnp-SNCA)M2Vle
• Allele Name: transgene insertion M2, Virginia M Y Lee
• Allele ID: MGI:5573154
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Tg(Cnp-MAPT*P301L)6Vle/0 Tg(Cnp-SNCA)M2Vle/0	involves: C3H * C57BL/6	MGI:5573158
tg2	Tg(Cnp-SNCA)M2Vle/Tg(Cnp-SNCA)M2Vle	involves: C3H * C57BL/6	MGI:5573172
tg3	Tg(Cnp-SNCA)M2Vle/0	involves: C3H * C57BL/6	MGI:5573159

Genotype
MGI:5573158

cx1

• Allelic Composition: Tg(Cnp-MAPT*P301L)6Vle/0; Tg(Cnp-SNCA)M2Vle/0
• Genetic Background: involves: C3H * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

behavior/neurological

jerky movement ( J:127923 )

• limb-twitch phenotype

nervous system

tau protein deposits ( J:127923 )

• mice exhibit accumulation of tau and alpha-synuclein amyloidogenic inclusions in oligodendrocytes as early as 12 months of age

alpha-synuclein inclusion body ( J:127923 )

• mice exhibit accumulation of tau and alpha-synuclein amyloidogenic inclusions in oligodendrocytes as early as 12 months of age

Genotype
MGI:5573172

tg2

• Allelic Composition: Tg(Cnp-SNCA)M2Vle/Tg(Cnp-SNCA)M2Vle
• Genetic Background: involves: C3H * C57BL/6

behavior/neurological

impaired coordination ( J:99814 )

• mice show a decrease in endurance time on the rotarod treadmill test beginning at 7-9 months of age

decreased grip strength ( J:99814 )

• mice show a progressive reduction in grip strength on a wire hanging test with advancing age

abnormal locomotor activation ( J:99814 )

• slow motor activity

nervous system

decreased brain weight ( J:99814 )

• 8% and 16% reduction in brain weight at 9 and 24 months of age, respectively

abnormal brain white matter morphology ( J:99814 )

• reduction in the white matter in 24 month old mice

brain atrophy ( J:99814 )

• 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

decreased oligodendrocyte number ( J:99814 )

• 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

gliosis ( J:99814 )

• in the brain and spinal cord

abnormal myelin sheath morphology ( J:99814 )

• degenerated myelin sheaths associated with degenerating axons in the CNS, most prominently in the spinal cord

decreased neuron number ( J:99814 )

• 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

neuron degeneration ( J:99814 )

• 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

abnormal spinal cord ventral horn morphology ( J:99814 )

• neuronal degeneration in the anterior horn of spinal cord

alpha-synuclein inclusion body ( J:99814 )

• 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

axon degeneration ( J:99814 )

• 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

demyelination ( J:99814 )

• demyelination of oligodendroctyes

mortality/aging

mortality/aging ( J:99814 )

N • life span is not affected

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
multiple system atrophy		DOID:4752		J:99814

Genotype
MGI:5573159

tg3

• Allelic Composition: Tg(Cnp-SNCA)M2Vle/0
• Genetic Background: involves: C3H * C57BL/6

nervous system

nervous system phenotype ( J:127923 )

N • mice do not develop tau or alpha-synuclein inclusions at 18 months of age