Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-Snca)1S13Putt mutation
(0 available)
Uchl1tm1Dgen mutation
(1 available);
any
Uchl1 mutation
(29 available)
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mortality/aging
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• at 3.5 months of age, mutants need to be sacrificed due to poor health
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nervous system
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• mice show a large increase in astrogliosis at 3.5 months of age compared to single Tg(Thy1-Snca)1S13Putt mutants, especially in the cortex and to a lesser extent in the hippocampus and the striatum, but not in the cerebellum, brainstem, and thalamus
• 3.5 month old mice however, do not show microgliosis or axon degeneration
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• increase in perikaryal alpha-synuclein accumulation in the cell soma of hippocampal neurons at 3.5 months of age
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behavior/neurological
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• mice show greater deficits in motor coordination on the rotarod than single Uchl1 homozygotes at 2.5 months of age
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• mice show greater deficits in muscle strength than single Uchl1 homozygotes at 2.5 months of age
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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nervous system
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• brainstem shows pronounced ubiquitin imunopathology
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• neurofilaments are short, thick, and less well oriented of about 10 nm in diameter, devoid of side-branches and coincide with non-fibrillar amorphous aggregates
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• central axons are often enlarged
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• ubiquitin immunoreactive motor neurons have spindle-shaped dilated proximal dendrites
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• neuromuscular synapses show signs of presynaptic degeneration that is independent of muscle fiber type
• neuromuscular junctions show thinning or absence of presynaptic neurofilaments
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• spinal cord shows pronounced ubiquitin imunopathology
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• ubiquitin immunoreactive motor neurons have spindle-shaped dilated proximal dendrites
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• alpha-synuclein non-fibrillar amorphous aggregates
(J:177661)
• multiple isoforms of alpha synuclein are seen, including serine 129 phosphorylated species in the most severely affected brain regions
(J:177661)
• neuronal serine 129 phosphorylated form of alpha-synuclein is seen in granular and small fibrillar aggregates and neurites show alternating segments of either alpha-synuclein or ubiquitin but not both
(J:177661)
• increase in perikaryal alpha-synuclein accumulation in the cell soma of hippocampal neurons
(J:207278)
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• axonal degeneration in long white matter tracts of the spinal cord with breakdown of myelin sheaths into rows of myelin ovoids
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• long white matter tracts of the spinal cord show breakdown of myelin sheaths into rows of myelin ovoids
• loosening of the myelin wraps and vesicular disruption of the myelin sheath
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behavior/neurological
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• around 6-7 months of age, mice start to display severe motor deficits, indicating late-onset motor impairment
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• during the first 4 weeks of age, mice show impaired motor learning on the accelerated rotarod task, but by 12 weeks of age and after a number of training sessions, performance is normal up to 6 months of age
• from 6-7 months of age onwards, mice show a steady and rapid decline in rotarod performance
• mice however show normal behavior in the open field, normal activity in the dark-light box and elevated plus maze, indicating no anxiety, and normal forelimb grip strength
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cellular
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• enlarged mitochondria with an abnormally high number of cristae without obvious vacuolization in spinal cord dendrites
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growth/size/body
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• around 6-7 months of age, mice stop gaining weight
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hematopoietic system
immune system
mortality/aging
muscle
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• muscles contain small angulated fibers reminiscent of neurogenic muscular atrophy
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