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Phenotypes Associated with This Genotype
Genotype
MGI:3603036
Allelic
Composition
Tg(Prnp-SNCA*A53T)83Vle/Tg(Prnp-SNCA*A53T)83Vle
Genetic
Background
involves: C3H * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-SNCA*A53T)83Vle mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

growth/size/body
• by 8 months of age, begin to lose weight (J:76657)
• by 8 months of age, begin to lose weight (J:76657)

behavior/neurological
• over time become unable to feed themselves (J:76657)
• over time become unable to feed themselves (J:76657)
• by 8 months of age, grooming is neglected (J:76657)
• by 8 months of age, grooming is neglected (J:76657)
• unable to right themselves when placed on their sides (J:76657)
• unable to right themselves when placed on their sides (J:76657)
• temulous motion is seen in some mice, possibly related to attempted muscular activity (J:76657)
• temulous motion is seen in some mice, possibly related to attempted muscular activity (J:76657)
• eventually are unable to stand up and support their own body weight (J:76657)
• eventually are unable to stand up and support their own body weight (J:76657)
• develop hunched backs by 8 months of age (J:76657)
• develop hunched backs by 8 months of age (J:76657)
• 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 (J:76657)
• 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 (J:76657)
• reduced ambulation by 8 months of age (J:76657)
• reduced ambulation by 8 months of age (J:76657)
• partial paralysis of limbs is observed by 8 months of age, beginning at a hindleg but affecting all limbs within a few days (J:76657)
• partial paralysis of limbs is observed by 8 months of age, beginning at a hindleg but affecting all limbs within a few days (J:76657)

nervous system
• astrocytic gliosis (J:76657)
• astrocytic gliosis (J:76657)
• endoneurial space is increased and axons are filled with vacuoles in the ventral roots of aged mice (J:76657)
• endoneurial space is increased and axons are filled with vacuoles in the ventral roots of aged mice (J:76657)
• 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 (J:76657)
• 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 (J:76657)
• show signs of neurodegeneration by 8 months of age and develop neurodegenerative disease within 16 months of age (J:76657)
• show signs of neurodegeneration by 8 months of age and develop neurodegenerative disease within 16 months of age (J:76657)
• significant axonal degeneration in aged mice (J:76657)
• significant axonal degeneration in aged mice (J:76657)
• following axonal degeneration, the myelin sheath loosens and unravels (J:76657)
• following axonal degeneration, the myelin sheath loosens and unravels (J:76657)

muscle
• exhibit sparse neurogenic muscle atrophy (J:76657)
• exhibit sparse neurogenic muscle atrophy (J:76657)

Mouse Models of Human Disease
OMIM ID Ref(s)
Parkinson Disease 1, Autosomal Dominant; PARK1 168601 J:76657


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory