Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr3tm1Jmi mutation
(2 available);
any
Egr3 mutation
(15 available)
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behavior/neurological
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• impaired gait that is less severe than in Runx3 null mice
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skeleton
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• develops between P40 and P90
• severity is reduced compared to Runx3 mice
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nervous system
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• superior cervical ganglion neurons grown in dissociated culture treated with NGF have fewer neurite branch points, decreased total neurite length and a reduction in length of the longest neurite compared to cultured wild-type neurons
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr3tm1Jmi mutation
(2 available);
any
Egr3 mutation
(15 available)
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mortality/aging
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• born in expected numbers but 40% are dead by weaning
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nervous system
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• at birth, mice exhibit a modest loss of all sizes of cholinergic motor neurons compared with wild-type mice
• at P5, mice exhibit increased motor neuron loss compared with wild-type mice due to loss of small diameter gamma-motor neurons
• at P10, loss of gamma-motor neurons is more complete with a 25% loss of gamma-motor neurons compared with wild-type mice
• at P20, mice exhibit a loss of small diameter cholinergic motor neurons compared with wild-type mice
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• at birth, mice exhibit a modest loss of all sizes of cholinergic motor neurons compared with wild-type mice
• at P5, mice exhibit increased motor neuron loss compared with wild-type mice due to loss of small diameter gamma-motor neurons
• at P10, loss of gamma-motor neurons is more complete with a 25% loss of gamma-motor neurons compared with wild-type mice
• at P20, mice exhibit a loss of small diameter cholinergic motor neurons compared with wild-type mice
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• number of muscle spindles is normal at birth but progressively disassemble
• only about 1/3 of normal numbers are present at 12 days of age and those are about 50% smaller than normal
• spindle response to stimulation is defective
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• adult skeletal muscles lack muscle spindles
• normal numbers of type I extrafusal fibers
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• 75% loss of large myelinated fibers and 90% loss of small myelinated fibers from the L4 dorsal root ganglion
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• normal numbers of proprioceptive neurons in newborn mice but the neurons are smaller in size
(J:49575)
• numbers of myelinated neurons drops to 75% of control numbers in adults
(J:76256)
• neuron loss is primarily type Ia and II sensory as well as gamma motor neurons
(J:76256)
• type Ib sensory neurons are unaffected
(J:76256)
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• type Ia neurons function normally during first week of life but synaptic connection to motoneurons is lost at 2 days of age
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• loss of monosynaptic excitatory postsynaptic potential normally generated by stimulation of proprioceptive neurons of muscles
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behavior/neurological
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• prominent resting tremors
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• abnormal limb position when stationary
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• waddling, uncoordinated gait
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muscle
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• number of muscle spindles is normal at birth but progressively disassemble
• only about 1/3 of normal numbers are present at 12 days of age and those are about 50% smaller than normal
• spindle response to stimulation is defective
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• adult skeletal muscles lack muscle spindles
• normal numbers of type I extrafusal fibers
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vision/eye
skeleton
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr3tm1Jmi mutation
(2 available);
any
Egr3 mutation
(15 available)
Egr3tm2.1Wgt mutation
(0 available);
any
Egr3 mutation
(15 available)
Tg(DBH-cre,-taulacZ)1Wgt mutation
(0 available)
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nervous system
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• conditional mutants display neuron loss in the superior cervical ganglion (SCG), similar to that described in germline Egr3 mutants
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• peripheral sympathetic target tissue innervation is abnormal in conditional mutants, closely resembling defects described in germline Egr3 mutants; innervation to the heart, spleen, kidney, and pineal gland is reduced
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vision/eye
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• sympathetic nervous system abnormalities like blepharoptosis are evident in conditional mutants, similar to defects described in germline Egr3 mutants
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behavior/neurological
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• mice lack the scoliosis and gait ataxia observed in Egr3 germline mutants
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nervous system
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• no significant differences are detected in sympathetic axon guidance; axon guidance to the submandibular gland is similar in mutants and controls
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• (lacZ-labeled) mutant neurons have fewer primary dendrites compared to controls in the superior cervical ganglion (SCG) and stellate ganglion; labeled neurons have decreased total dendrite length and decrease in maximum extent of the dendritic arbor in the SCG
• neurons are significantly atrophic compared with control (decreased mean soma area)
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr3tm1Jmi mutation
(2 available);
any
Egr3 mutation
(15 available)
Gfra1tm3Jmi mutation
(0 available);
any
Gfra1 mutation
(31 available)
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nervous system
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• mice exhibit selective loss of small cholinergic neurons unlike wild-type mice
• total numbers of cholinergic neurons in the L4-5 section of the spinal cord is decreased compared to in wild-type mice
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• total numbers of cholinergic neurons in the L4-5 section of the spinal cord is decreased compared to in wild-type mice
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr3tm1Jmi mutation
(2 available);
any
Egr3 mutation
(15 available)
Gfra1tm3Jmi mutation
(0 available);
any
Gfra1 mutation
(31 available)
Tg(Hlxb9-GFP)1Tmj mutation
(3 available)
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nervous system
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• mice exhibit a loss of small diameter cholinergic motor neurons compared with wild-type mice
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• surviving Gfra1+ motor neurons exhibit somatic shrinkage and morphological evidence of degeneration unlike in wild-type mice
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• Gfra1+, cholinergic, Gfa1+ HB9-GFP-, and Gfra1+ HB9-GFP+ motor neurons are reduced compared to in wild-type mice
• mice exhibit a loss of small diameter cholinergic motor neurons compared with wild-type mice
• however, the number of large diameter motor neurons is normal
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• surviving Gfra1+ motor neurons exhibit somatic shrinkage and morphological evidence of degeneration unlike in wild-type mice
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Analysis Tools