Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp13a2tm1.2Wtd mutation
(0 available);
any
Atp13a2 mutation
(60 available)
Tg(Prnp-SNCA*A53T)83Vle mutation
(1 available)
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cellular
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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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nervous system
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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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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sncatm1Rosl mutation
(4 available);
any
Snca mutation
(34 available)
Tg(Prnp-SNCA*A53T)83Vle mutation
(1 available)
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nervous system
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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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immune system
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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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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-SNCA*A53T)83Vle mutation
(1 available)
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mortality/aging
growth/size/body
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• by 8 months of age, begin to lose weight
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behavior/neurological
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• over time become unable to feed themselves
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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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• eventually are unable to stand up and support their own body
weight
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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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nervous system
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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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muscle
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• exhibit sparse neurogenic muscle atrophy
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limbs/digits/tail
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• exhibit sparse neurogenic muscle atrophy
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-SNCA*A53T)83Vle mutation
(1 available)
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behavior/neurological
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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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nervous system
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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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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-SNCA*A53T)83Vle mutation
(1 available)
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behavior/neurological
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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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homeostasis/metabolism
nervous system
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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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taste/olfaction
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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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Analysis Tools