normal phenotype
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• homozygotes are viable, fertile, grow at a normal rate, and show no histological or physiological abnormalities at >1 year of age
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hm1
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Mettm1Sst/Mettm1Sst
involves: 129P2/OlaHsd * C57BL/6 |
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• homozygotes are viable, fertile, grow at a normal rate, and show no histological or physiological abnormalities at >1 year of age
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cn2
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Mettm1Cpo/Mettm1Sst Tg(Nes-cre)1Kln/? involves: (129P2/OlaHsd * 129S1/Sv)F1 * 129X1/SvJ * C57BL/6 * SJL |
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| N |
• no effect on retinal ganglion cell survival after optic nerve axotomy
(J:173661)
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cn3
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Mettm1Sst/Mettm1Sst Tg(SFTPC-rtTA)5Jaw/0 Tg(tetO-cre)1Jaw/0 involves: 129 * 129P2/OlaHsd * C57BL/6 |
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• newborn mice exposed to doxycycline from E14.5 to birth display defective distal lung saccular development due to reduced primary septation
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• newborn mice exposed to doxycycline from E14.5 to birth display dilated distal airspaces with only few primary septae, unlike wild-type controls where extensive numbers of saccules are observed
|
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cn4
|
Mettm1Sst/Mettm1Sst Trp53tm1Brn/Trp53tm1Brn involves: 129P2/OlaHsd |
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| N |
• ovarian surface epithelium cells transfected with a cre-expressing adenovirus exhibit reduced invasion compared with cells from Trp53tm1Brn homozygotes transfected with a lacZ-expressing adenoviru
(J:175978)
|
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cn5
|
Mettm1Cpo/Mettm1Sst Tg(Nes-cre)1Kln/0 involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL |
|
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• mice exhibit reduced motor axon arborization (length and number of branches) in the pectoralis minor muscle compared with wild-type mice
• at P2, endplates in the pectoralis minor exhibit increased partially or completely denervated synapses compared with wild-type endplates
• however, mice exhibit normal innervation of the pectoralis minor muscle at E15.5 and of the pectoralis major
|
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• by P2 in the C8 and T1 region of the spinal cord with no further reduction in adult mice
|
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• mice exhibit reduced motor axon arborization (length and number of branches) in the pectoralis minor muscle compared with wild-type mice
• at P2, endplates in the pectoralis minor exhibit increased partially or completely denervated synapses compared with wild-type endplates
• however, mice exhibit normal innervation of the pectoralis minor muscle at E15.5 and of the pectoralis major
|
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• in a wire hang test but not a rotarod
|
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cn6
|
Mettm1Sst/Mettm1Sst Trp53tm1Brn/Trp53tm3Tyj involves: 129P2/OlaHsd * 129S4/SvJae |
|
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| N |
• ovarian surface epithelium cells transfected with a cre-expressing adenovirus exhibit reduced invasion compared with similarly treated cells from Trp53tm1Brn/Trp53tm3Tyj mice
(J:175978)
|
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cn7
|
Mettm1Sst/Mettm1Sst Trp53tm1Brn/Trp53tm2Tyj involves: 129P2/OlaHsd * 129S4/SvJae |
|
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| N |
• ovarian surface epithelium cells transfected with a cre-expressing adenovirus exhibit reduced invasion compared with similarly treated cells from Trp53tm1Brn/Trp53tm2Tyj mice
(J:175978)
|
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cn8
|
Mettm1Sst/Mettm1Sst Tg(Alb-cre)21Mgn/0 involves: 129P2/OlaHsd * C57BL/6 |
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• mutants develop hemorrhagic liver destruction within the first few hours in response to apoptotic stimuli induced by Fas (Tnfrsf6) antibody compared to only minor liver damage in controls
|
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• primary hepatocytes show reduced phagocytic activity towards bacteria (E.coli) than control cells
|
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• hepatocytes exhibit increased sensitivity to Fas (Tnfrsf6)-induced apoptosis
|
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• the ability of mutant livers to clear necrotic tissue and to repopulate the injured (by CCl4 exposure) area by regenerating hepatocytes is impaired
• associated with a persistent inflammatory reaction, overproduction of osteopontin, early and prominent dystrophic calcification, and impaired hepatocyte scattering/migration into diseased areas
|
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• 80% of mutants die of acute liver failure in response to apoptotic stimuli induced by Fas (Tnfrsf6) antibody while controls survive
|
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• mutants develop hemorrhagic liver destruction within the first few hours in response to apoptotic stimuli induced by Fas (Tnfrsf6) antibody compared to only minor liver damage in controls
|
|
• mutants exhibit delayed healing after toxic liver injury induced by CCl4 exposure
• mutants are more vulnerable to hepatectomy surgery and most are either moribund or die by 48 hours after surgery
|
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• hepatocytes fail to migrate in a standard wound healing assay
|
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• hepatocytes exhibit increased sensitivity to Fas (Tnfrsf6)-induced apoptosis
|
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cn9
|
Mettm1Ics/Mettm1Sst Tg(Nes-cre)1Kln/0 involves: 129P2/OlaHsd * C57BL/6 * SJL |
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|
• by P2 with no further reduction in adult mice
• lacZ+ motor neurons are reduced at P2 in C8 and T1 regions of the spinal cord
|
|
cn10
|
Mettm1Sst/Mettm1Sst Tg(mI56i-cre,EGFP)1Kc/0 involves: 129P2/OlaHsd * C57BL/6J * FVB |
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| N |
• in the Morris water maze, mutants have a longer latency to locate the platform on the first 2 days of training than controls, however performance on the probe test and reversal probe test were normal, indicating normal hippocampal-mediated spatial learning
(J:170554)
• open field activity and anxiety are not affected
(J:170554)
|
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• on reversal discriminations, mutants require more trials than controls, indicating impaired reversal learning
|
|
• mutants show increased latency performance on the cued-platform test, indicating a delay in striatal-dependent cued learning
|
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• marker analysis indicates an increase in numbers of PV+ and SST+ striatal GABAergic interneurons
• distribution of striatal interneurons is altered, with fewer cells of the population located in the medial (associative) regions and more cells in the lateral (sensorimotor) areas
• reduction in the numbers of PV+ GABAergic interneurons in the primary somatosensory cortex (28% loss) and the orbitofrontal cortex (31% loss)
|
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• marker analysis indicates an increase in numbers of PV+ and SST+ striatal GABAergic interneurons
• however, striatum appears normal
|
|
• 31% reduction in PV+ cells in the orbitofrontal cortex
|
|
• 28% loss of PV+ cells in primary somatosensory cortex
|
Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Autism, Susceptibility to, 9; AUTS9 | 611015 | J:170554 | |
| Gilles De La Tourette Syndrome; GTS | 137580 | J:170554 | |