Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Mapttm2(FUS)Neas mutation
(0 available);
any
Mapt mutation
(428 available)
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nervous system
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• motor neuron loss is seen at P60 but not P30, when 10% of lumbar level 5 (L5) motor neurons are lost and by P360, there are 18.6% fewer motor neurons overall in the L5 segment
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• about 10% denervation of the tibialis anterior neuromuscular junctions (NMJs), first seen at P60 which progresses with age such that at P360, about 25% of NMJs are denervated
• denervation is first noted at P120 in the gastrocnemius muscle where about 10% of NMJs are denervated
• however, even at P360, no denervation is seen in the soleus muscle NMJs
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Mapttm3(FUS)Neas mutation
(0 available);
any
Mapt mutation
(428 available)
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nervous system
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• significant motor neuron loss is seen at P30 but not P10, and by P360, there are 22.6% fewer motor neurons overall in the lumbar level 5 (L5) segment
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• about 10% denervation of the tibialis anterior neuromuscular junctions, first seen at P20 which progresses with age such that at P360, about 30% of NMJs are denervated
• denervation is first noted at P120 in the gastrocnemius muscle where about 13% of NMJs are denervated
• however, even at P360, no denervation is seen in the soleus muscle NMJs
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Mapttm3(Sema3e)Arbr mutation
(0 available);
any
Mapt mutation
(428 available)
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nervous system
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• 23% of triceps motor neurons receive monosynaptic input from triceps afferents compared to 95% in wild-type mice
• however, triceps and cutaneous maximus motor neurons normally lack monosynaptic input from cutaneous maximus afferents
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behavior/neurological
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N |
• mice exhibit no behavior deficits
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respiratory system
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• abnormal hypoxic breathing response
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behavior/neurological
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N |
• mice exhibit normal activity
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cardiovascular system
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N |
• mice exhibit a regular heart rate and show rescue of spontaneous and induced cardiac arrhythmias, long QT, and improved survival
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homeostasis/metabolism
nervous system
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• starburst amacrine cells in flat mount retinas show excess and aberrant light-evoked inhibitory postsynaptic currents
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mortality/aging
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• 67% of mice die early with a median life span of 16 months
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cardiovascular system
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• irregular heart rhythm and sudden drops in heart rate
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• increase in heart rate variability during both the light and dark cycles
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• heart rate during the dark cycle is decreased but is not different during the light cycle
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• mice show an increased rate of spontaneous ventricular arrhythmias including premature ventricular contractions and nonsustained ventricular tachycardia
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• mice show an increased rate of spontaneous ventricular arrhythmias including premature ventricular contractions and nonsustained ventricular tachycardia
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• mice are highly susceptible to inducible arrhythmias
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homeostasis/metabolism
behavior/neurological
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N |
• mice show no differences in activity during the light or dark cycle
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respiratory system
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N |
• mice do not exhibit increased basal apneas or abnormal hypoxic response
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nervous system
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• at P0, presynaptic and postsynaptic terminal distribution is diffuse and nerve terminal sprouting occurs unlike in wild-type mice that is not as severe as in Aplp2tm1Dbo Apptm1.2Zhe double homozygotes
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Fustm1a(EUCOMM)Wtsi mutation
(0 available);
any
Fus mutation
(39 available)
Fustm1c(EUCOMM)Wtsi mutation
(0 available);
any
Fus mutation
(39 available)
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nervous system
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N |
• no motor neuron degeneration is seen up to 1 year of age
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Stmn2em2Jmi mutation
(0 available);
any
Stmn2 mutation
(41 available)
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mortality/aging
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• 16.8% of mice are obtained at P21, indicating a significant deviation from Mendelian ratios
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behavior/neurological
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• at 3 months of age, mice show a significant decrease in the latency time (s) to fall from an inverted screen relative to control mice
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nervous system
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• mice develop a distal motor neuropathy
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• at 3 months of age, lumbrical muscles show a NMJ phenotype similar to that of single constitutive Stmn2em1Jmi homozygotes, where most lumbrical muscle fibers are partially denervated with highly fragmented postsynaptic endplates
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vision/eye
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• the starburst amacrine cell (SAC) processes that normally stratify in 2 discrete inner plexiform laminae (S2 and S4) fail to completely segregate from each other
• disruption of the dendritic plexus organization of On but not Off SACs
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• the starburst amacrine cell (SAC) processes that normally stratify in 2 discrete inner plexiform laminae (S2 and S4) fail to completely segregate from each other
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nervous system
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• the starburst amacrine cell (SAC) processes that normally stratify in 2 discrete inner plexiform laminae (S2 and S4) fail to completely segregate from each other
• disruption of the dendritic plexus organization of On but not Off SACs
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• the starburst amacrine cell (SAC) processes that normally stratify in 2 discrete inner plexiform laminae (S2 and S4) fail to completely segregate from each other
• disruption of the dendritic plexus organization of On but not Off SACs
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chattm2(cre)Lowl mutation
(4 available);
any
Chat mutation
(57 available)
Cpeb2tm1.1Yshu mutation
(0 available);
any
Cpeb2 mutation
(35 available)
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respiratory system
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• whole-body plethysmography revealed respiratory defects similar to those observed in Cpeb2tm1.2Yshu homozygotes but with wide individual variation in severity
• however, tidal volume is normal at P1
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• aberrant respiration patterns at P1
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• reduced respiratory frequency at P1
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• increased apneic episodes at P1
• inhalation of nebulized tiotropium, an anticholinergic bronchodilator, reduces apnea frequency to wild-type levels
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• significantly increased methacholine-induced airway reactivity in adult mice
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homeostasis/metabolism
Analysis Tools