Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Prnp-SNCA*A53T)83Vle transgene insertion 83, Virginia M-Y Lee MGI:3057167 |
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| Summary |
5 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice show a similar level of lysosomal accumulation as seen in single Atp13a2 homozygotes at 3 and 9 months of age
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• mice show similar levels of gliosis at 3 months of age and decreased levels of gliosis at 9 months compared to single Atp13a2 homozygotes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• following injection of LPS into the substantia nigra
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• following injection of LPS into the substantia nigra, mice exhibit an increased loss of dopaminergic neurons that is associated with insoluble and aggregated SNCA compared to in Sncatm1Rosl Tg(Prnp-SNCA)7Vle and Sncatm1Rosl homozygotes
• however, loss of dopamine neurons is not due to an abnormal inflammatory response following injection of LPS into the substantia nigra
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• following injection of LPS into the substantia nigra, mice exhibit an increased loss of dopaminergic neurons compared to in Sncatm1Rosl Tg(Prnp-SNCA)7Vle and Sncatm1Rosl homozygotes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• by 8 months of age, begin to lose weight
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• by 8 months of age, grooming is neglected
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• unable to right themselves when placed on their sides
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• temulous motion is seen in some mice, possibly related to attempted muscular activity
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• develop hunched backs by 8 months of age
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• by 8 months of age, exhibit severe movement impairment with resistance to passive movement and partial paralysis of limbs, accompanied by periods of freezing of hindlimb
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• reduced ambulation by 8 months of age
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• partial paralysis of limbs is observed by 8 months of age, beginning at a hindleg but affecting all limbs within a few days
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• endoneurial space is increased and axons are filled with vacuoles in the ventral roots of aged mice
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• develop age-dependent intracytoplasmic neuronal alpha-synuclein inclusions that contain 10-16 nm wide fibrils similar to those seen in human alpha-synucleinopathies, with dense accumulation in the spinal cord, brainstem, cerebellum, and thalamus
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• show signs of neurodegeneration by 8 months of age and develop neurodegenerative disease within 16 months of age
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• significant axonal degeneration in aged mice
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• following axonal degeneration, the myelin sheath loosens and unravels
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• exhibit sparse neurogenic muscle atrophy
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• exhibit sparse neurogenic muscle atrophy
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Parkinson's disease 1 | DOID:0060367 |
OMIM:168601 |
J:76657 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• develop the severe and complex motor impairment leading to paralysis and death that is seen in homozygous transgenics, however onset is delayed from 16 months of age to 22-28 months of age
(J:76657)
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• develop a similar neurodegenerative disease that is observed in homozygous transgenic mice, however onset is delayed from 16 months of age to 22-28 months of age
(J:76657)
• mice develop some inclusions that are composed of both tau and alpha-synuclein
(J:127923)
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• about 25% of diseased mutants accumulate abundant tau-positive threads, grains and spheroids in regions of the pons, midbrain, and spinal core
• rare perikaryal tau inclusions with morphology of a pre-tangle are seen
• less frequent tau inclusions are present in another 25% of symptomatic mutants but none are seen in the rest 50% of diseased mutants
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• mice exhibit formation of abundant alpha-synuclein inclusions
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Parkinson's disease 1 | DOID:0060367 |
OMIM:168601 |
J:76657 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• 6 and 10 month, but not 3 month, old mice do not sniff novel scent more than familiar scent
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• decrease in acetylcholinesterase activity in olfactory bulb in 6 and 10 month old mice
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• increase in dopaminergic neurons in olfactory bulb glomerular layer in 10 month old mice
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• decreased numbers of cholinergic neurons are found in the olfactory bulb mitral layer in 10 month old mice
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• increase in dopaminergic neurons in olfactory bulb glomerular layer in 10 month old mice
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• decreased numbers of cholinergic neurons are found in the olfactory bulb mitral layer in 10 month old mice
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• time to locate buried food is increased in 6 and 10 month, but not 3 month, old mice as compared to wild-type
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 10/29/2024 MGI 6.24 |
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