growth/size
| N |
• body weigh does not differ from controls measured out to 18 months of age
(J:156741)
|
behavior/neurological
|
• males and females exhibit consistently reduced latency to fall beginning at 6 months; this persisted at 1 year of age and more pronounced at 18 months; endurance and coordination in the rotating rod test are abnormal
|
hypoactivity
(
J:156741
)
|
• total distance traveled in the open field apparatus is reduced compared to controls at 6 months and remains abnormal at 18 months
• distance traveled decreases with age, showing reduced ambulatory distance traveled compared to controls at 12 and 18 months; reduction is generalized, not specific to a particular type of movement or region
|
nervous system
| N |
• dorsal motor nucleus of the vagus shows no abnormal alpha-synuclein aggregation and any widespread aggregates are not detected in brain homogenates of 5 and 18 month-old animals
(J:156741)
• striatal tissue dopamine and dopamine metabolite content are not different from controls at 11 and 18 months
(J:156741)
• no progressive loss of dopaminergic neurons in the substantia nigra, pars compacta (SNpc) is observed
(J:156741)
• olfaction and cardiac innervation by the autonomic nervous system are not affected by the transgenes
(J:156741)
|
|
• some dystrophic synapses are observed in rare instances at 12 or 22 months
|
|
• protein is present in tyrosine hydroxylase (TH)-immunoreactive neuronal cell bodies within myenteric and submucosal plexuses; varicose TH-positive terminals of noradrenergic sympathetic neurons
• most alpha-synuclein positive neurons, especially in the myenteric plexus are not coincident with TH immunostaining
• proteinase K-resistant alpha-synuclein aggregates are observed in the nuclear and perinuclear cytoplasm of enteric nervous system neurons in mice showing gastrointestinal motility dysfunction
|
digestive/alimentary system
|
• proteinase K-resistant alpha-synuclein aggregates are observed in the nuclear and perinuclear cytoplasm of enteric nervous system neurons in mice showing gastrointestinal motility dysfunction
|
|
• whole-gut transit time (WGTT) is prolonged compared to controls starting at 3 months and is markedly prolonged at 6 months persisting through 18 months of age; this phenotype does not differ between males and females in contrast to bead expulsion time (distal colonic motility)
|
|
• motility of distal colon is reduced compared to controls with males showing around a 4-5 fold prolongation in expulsion compared to controls; reduction is exaggerated compared to females
• this phenotype is present at 3 months and persists through 18 months of age
• amount of stool passed/unit time and stool water content are unchanged at 3 months of age but by 6 months these parameters are reduced to comparable degrees
|
cardiovascular system
| N |
• no defects in autonomic cardiac innervation are detected
(J:156741)
|
taste/olfaction
| N |
• mice show no impairments in olfaction at 12 and 18 months of age
(J:156741)
• olfactory bulbs show no degeneration or protein aggregation at 12 and 18 months of age
(J:156741)
|
Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Parkinson Disease 1, Autosomal Dominant; PARK1 | 168601 | J:156741 | |
