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Allele Symbol Allele Name Allele ID |
Grntm1.1Far targeted mutation 1.1, Bob Farese MGI:4949110 |
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| Summary |
3 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
Increased total and phosphorylated TDP-43 in the cytoplasm of thalamic neurons in Grntm2.1Far/Grntm2.1Far and Grntm1.1Far/Grntm1.1Far mice
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• mice exhibit increased levels of total and phosphorylated TDP-43 in the cytoplasm of thalamic neurons
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• mice exhibit increased levels of total and phosphorylated TDP-43 in the cytoplasm of thalamic neurons
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• increased 3-fold in MPTP-treated mice
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• from microglia exposed to LPS and IFN-gamma
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• from microglia exposed to LPS and IFN-gamma
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• from microglia exposed to LPS and IFN-gamma
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• from microglia exposed to LPS and IFN-gamma
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• levels of nuclear TDP-43 is reduced in retinal ganglion cell layer neurons of 18 month old mutants, whereas levels of cytoplasmic TDP-43 are unchanged
• depletion of nuclear TDP-43 also occurs in 12 month old mice before significant retinal ganglion cell layer neuron loss
• neither nuclear nor cytoplasmic TDP-43 inclusions are seen in retinal ganglion cell layer neurons
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• loss of ganglion cell layer cells in 18 month old mutants
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• progressive thinning of the retinal nerve fiber layer
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• age-dependent reduction in a wave amplitude
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• age-dependent reduction in b wave amplitude
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• mutants have reduced amplitudes of photopic negative responses at 18, but not 12, months of age
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• increased 3-fold in MPTP-treated mice
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• greater in mice treated with MPTP compared with control mice
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• levels of nuclear TDP-43 is reduced in retinal ganglion cell layer neurons of 18 month old mutants, whereas levels of cytoplasmic TDP-43 are unchanged
• depletion of nuclear TDP-43 also occurs in 12 month old mice before significant retinal ganglion cell layer neuron loss
• neither nuclear nor cytoplasmic TDP-43 inclusions are seen in retinal ganglion cell layer neurons
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• retinal neurodegeneration
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• loss of ganglion cell layer cells in 18 month old mutants
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• culture media conditioned with microglia exposed to LPS and IFN-gamma can induce increased cell death in wild-type neurons
(J:188324)
• primary cortical neurons grown in culture exhibit shorter survival than wild-type neurons
(J:218705)
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• induced by MPTP
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• induced by MPTP
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• induced by MPTP
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• increased 3-fold in MPTP-treated mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Grn-related frontotemporal lobar degeneration with Tdp43 inclusions | DOID:0060672 |
OMIM:607485 |
J:218705 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• cultured peritoneal macrophages engulf latex beads more rapidly than similarly treated wild-type cells
• cultured peritoneal macrophages have increased levels of phagocytosis of yeast cell wall particles and apoptotic thymocytes compared to wild-type cells
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• cultured peritoneal macrophages engulf latex beads more rapidly than similarly treated wild-type cells
• cultured peritoneal macrophages have increased levels of phagocytosis of yeast cell wall particles and apoptotic thymocytes compared to wild-type cells
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 04/16/2024 MGI 6.23 |
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