Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ei24tm1Hzha mutation
(0 available);
any
Ei24 mutation
(63 available)
Speer6-ps1Tg(Alb-cre)21Mgn mutation
(6 available);
any
Speer6-ps1 mutation
(4 available)
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Hepatomegaly and tumor-like protrusions in the liver of Ei24tm1Hzha/Ei24tm1Hzha Speer6-ps1Tg(Alb-cre)21Mgn/0 mice
homeostasis/metabolism
liver/biliary system
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• by 10 months, livers exhibit multiple tumor-like protusions with no signs of malignancy
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• disorganized hepatic lobules
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• swollen hepatocytes with swollen mitochondria
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growth/size/body
cellular
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• impaired autophagic flux in the liver at 3 months
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ei24tm1Hzha mutation
(0 available);
any
Ei24 mutation
(63 available)
Tg(Nes-cre)1Kln mutation
(4 available)
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Limb-clasping in Ei24tm1Hzha/Ei24tm1Hzha Tg(Nes-cre)1Kln/0 mice
homeostasis/metabolism
mortality/aging
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• 50% of mice die by 16 weeks
• all mice die by 19 weeks
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nervous system
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• mice do not exhibit accumulation of TDP-43 (Tardbp) aggregates in motor neurons
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• neural abnormalities increase progressively with age
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• thin white matter between the cortex and hippocampal pyramidal cells
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• reduced ratio of cortical to dorsoventral thickness
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• vacuolated cells in the cingulate cortex and to a lesser extent in other cortices, hippocampus, thalamus, and hypothalamus
• vacuolated cells in the white matter regions including the corpus callosum, the alveus of the hippocampus and the internal capsule
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• in the cerebrum, cerebellum and spinal cord
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• numerous vacuolated cells in the spinal cord gray and white matter
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• irregularly arranged in the alveus of the hippocampus
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• thinned and split in the deep cerebellar nuclei axons
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• in cortical layers 1 through 4
• reduced interneuron numbers in the anterior horn of the lumbar spinal cord
• however, the number of motor neurons is normal
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• in the fifth layers of the motor and sensory cortices
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• in the cerebrum, cerebellum and spinal cord that increase in number and size with age
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• massive
• of Purkinje cell axons
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• of Purkinje cell axon terminals
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• eosinophilic spheroids in the cortex
• large eosinophilic spheroids in the deep cerebellar nuclei
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behavior/neurological
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• motor and behavioral deficits beginning at 6 weeks and progressively worsening
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• beginning at 6 weeks and progressively worsening
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• hyperextended position of hind limbs
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• in 8 of 12 mice at 6 weeks
• in all mice at 8 weeks
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growth/size/body
cellular
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• impaired autophagic flux in the brain and spinal cord at 4 months
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