Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Erbb2tm1Cbm mutation
(0 available);
any
Erbb2 mutation
(59 available)
Erbb2tm2Cbm mutation
(0 available);
any
Erbb2 mutation
(59 available)
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mortality/aging
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• 5 of 37 die within 6 months
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behavior/neurological
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• difficulties in hindlimb movement
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growth/size/body
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• display wasting associated with weight loss and premature death
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limbs/digits/tail
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• kinked or serpentine tails
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nervous system
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• at P3.5, see a 10-fold reduction in ventral and a 5-fold reduction in dorsal roots, with this reduction still apparent at P15, however by 6 months of age, cell numbers are similar to those in controls, however myelin sheaths formed are thin
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• reduction in thickness of the myelin sheath of sciatic nerves at P15 that persists at 6 and 14 months of age
• thin myelin is due to the presence of fewer wraps of myelin around the axon
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• dorsal roots display a severe decrease of Schwann cells in all mutants at P3.5 and P15
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• sciatic nerves at P15 are translucent and thin
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• ventral roots display a severe decrease of Schwann cells in all mutants at P3.5 and P15
• about 20% loss of axons in the L5 ventral root
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• hypomyelination in sciatic, sural, saphenous nerves, nerves innervating lower leg muscles, and the dorsal spinal roots and absent myelin in ventral spinal roots
• occasionally see large-caliber axons lacking myelin that are still surrounded by a Schwann cell basal lamina in the sciatic nerve
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Erbb2tm2Cbm mutation
(0 available);
any
Erbb2 mutation
(59 available)
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nervous system
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• peripheral without Schwann cell turn-over
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behavior/neurological
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N |
• mice exhibit normal motor coordination
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nervous system
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• mice show normal myelination at P15, however axons of peripheral nerves are hypermyelinated by P90
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nervous system
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• myelination defects are more pronounced than in single conditional Ptpn11tm1.1Wbm homozygotes
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nervous system
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• in the ventral but not dorsal roots
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• peripheral nerves are hypomyelinated with thinner myelin in axons with larger axon diameter
(J:153219)
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cacna1dtm1Hgn mutation
(0 available);
any
Cacna1d mutation
(119 available)
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
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hearing/vestibular/ear
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• above 60 dB, the amplitudes of waves II and III are increased compared to in control mice
• between 50 and 75 dB, wave I amplitude if slightly decreased compared to in control mice
• however, mice exhibit normal ABR thresholds for pure tone stimuli and in response to click or noise burst stimuli
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• slightly reduced at 50 dB SPL f2
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• slightly increased threshold in the middle hearing range
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nervous system
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• the lateral superior olive is round not U shape as in control mice and reduced in volume due to reduced neuron number
• the medial nucleus of the trapezoid body is reduced in volume due to reduced neuron number compared to in control mice
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• in the lateral superior olive and medial nucleus of the trapezoid body
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Gt(ROSA)26Sortm5(CAG-EGFP,-lacZ)Dym mutation
(0 available);
any
Gt(ROSA)26Sor mutation
(942 available)
Tg(Fev-flpe)1Dym mutation
(0 available)
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normal phenotype
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• alleles are used in intersectional and subtractive cell lineage fate mapping to visualize serotonin (5-HT) producing neurons and their precursors in the E12.5 embryonic hindbrain or mature brainstem
• rhombomere-3 (r3) rhombomere-5 (r5) derived 5-HT precursor cells are labeled by beta-gal while 5-HT precursors arising outside of r3 or r5 are labeled by eGFP
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Mnat1tm2.1Tpm mutation
(0 available);
any
Mnat1 mutation
(26 available)
Mnat1tm2Tpm mutation
(0 available);
any
Mnat1 mutation
(26 available)
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reproductive system
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• at 6 weeks of age, pyknotic germ cells in the seminiferous tubules in testis indicative of spermatogonia and spermatocyte cell death
• at 10 weeks of age, severe wasting and dramatically reduced cellularity was visible macroscopically; entirely devoid of all germ cells and their derivatives
• at 3 weeks of age, the developing germ lineage in the seminiferous tubules was completely indistinguishable from control animals
• at 10 weeks of age, Sertoli cells in the seminiferous tubules and cells of interstitium were normal
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behavior/neurological
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• exhibit a variety of symptoms of Schwann cell dysfunction including gait abnormalities beginning at 3 month of age
• mutant were viable and healthy and displayed no signs of neuropathy or myelin dysfunction into early adulthood up to 3 month of age
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growth/size/body
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• beginning at 3 month of age, pronounced in the hind limbs suggestive of neuropathy and subsequent neurogenic muscular atrophy
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nervous system
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• at 3 months of age, the mutant exhibited a marked increase in Schwann lineage proliferation
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• beginning at 3 month of age, large diameter axons completely denuded of myelin are present in sciatic nerves
• by 5 month of age, essentially all of the myelinated axons shows signs of extensive demyelination
• sciatic nerves from mutant animals at 1 month and 2 month of age showed normal myelin thickness compared to axonal diameter indicating myelinating Schwann cells are capable of attaining and a mature myelinated phenotype
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Robo3tm1.1Ache mutation
(0 available);
any
Robo3 mutation
(71 available)
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Reduced internuclear commissure in Robo3tm1.1Ache/Robo3tm1.1Ache Egr2tm2(cre)Pch/Egr2+ mice
nervous system
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• at E11 - E12, a severe reduction of commissural projections in r3 and r5 is seen
• at E15 and in newborns, virtually no axons are found to cross the midline at the level of the abducens nuclei
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• globular bushy cell projections from the anteroventral cochlear nucleus to the principal cells of the median nucleus of the trapezoid body are exclusively ipsilateral
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behavior/neurological
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• impairment in horizontal visual vestibuloocular reflex (VVOR) but not in vertical VVOR
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• impairment in horizontal OKR but not in vertical OKR
• defects occur predominantly in the low frequency range
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• impairment in horizontal VOR but not in vertical VOR
• defects in VOR are more predominant at high frequencies
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hearing/vestibular/ear
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• impairment in horizontal VOR but not in vertical VOR
• defects in VOR are more predominant at high frequencies
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• 3 rather than 4 waves are present ipsilaterally and the third wave has a longer latency and is not followed by any identifiable wave
• the appearance of contralateral wave IVc is delayed compared to controls
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Gt(ROSA)26Sortm7(Pik3ca*,EGFP)Rsky mutation
(1 available);
any
Gt(ROSA)26Sor mutation
(942 available)
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nervous system
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• myelination is impaired
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Gt(ROSA)26Sortm8(Map2k1*,EGFP)Rsky mutation
(1 available);
any
Gt(ROSA)26Sor mutation
(942 available)
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nervous system
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• onion bulb structures are seen indicating myelin degeneration and regeneration
• however, stacking of myelin sheaths in compact myelin is unaffected
• noncompact myelin structures are more abundant
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• myelin sheaths are thicker at P15 and P30 and myelin continues to grow radially such that the mean myelin area in peripheral nerves at P90 is increased 6-fold
• increase in myelin thickness is particularly pronounced in small-diameter fibers
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• aberrant paranodal myelin (tomaculi) and infolded myelin loops
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• increase in the diameter of sciatic nerves due to massive hypermyelination and reduced axonal packing
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• myelination begins prematurely and mice show continuous myelin growth
• increase in the number of myelinated fibers per field at P0
• myelin abnormalities are seen at P30-P90 but not at P15, indicating that they result from massive hypermylination
• treatment with the mTORC1 inhibitor everolimus dampens myelin growth in the nerves of mutants and completely rescues the decreased axonal packing
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• ratio of compound action potentials in myelinated A and nonmyelinated C fibers (A/C fiber waves) is reduced and there is a reduction in numbers of active mechanoreceptive units after P60 indicating that many myelinated axons are not conducting
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• D-hair mechanoreceptors show increased conduction velocities at P30, however no differences were seen after P60
• however, conduction velocities of other mechanosensory fibers are unchanged
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
Robo3tm1.1Ache mutation
(0 available);
any
Robo3 mutation
(71 available)
Tg(Mnx1-EGFP)#Tmj mutation
(0 available)
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nervous system
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• at E14, the abducens motor nucleus is closer to the floor plate and abnormally shaped
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mortality/aging
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• 1 week after birth, mice are dead
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nervous system
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• mice exhibit impaired development of retrotapezoid nucleus neurons compared with wild-type mice
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• the parafacial area e-pF oscillator exhibits only occasional motor activity bursts with reduced frequency compared to in wild-type mice
• rhythmic phrenic discharges are less frequent than in wild-type mice
• mice fail to exhibit an accelerated respiratory-like rhythm phrenic discharge in response to low pH challenge unlike similarly treated wild-type mice
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cellular
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• mice exhibit impaired development of retrotapezoid nucleus neurons compared with wild-type mice
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mortality/aging
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N |
• the great majority of mice survive to adulthood
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nervous system
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• massive depletion of retrotrapezoid nucleus neurons at P9
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• frequency of rhythmic phrenic discharges is reduced and does not respond to acidification
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respiratory system
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• hypoxic response is more vigorous (increase in ventilation is more pronounced and lasts longer) compared to controls
• exposure to pure O2 triggers periodic breathing in mutants but not in controls
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• in normoxia mean ventilation is reduced due to longer breath duration
• complete absence of normal response to hypercapnia at P2 and P9
• at 3 weeks and 4 months of age mice display a reduced increase in ventilation in response to hypercapnia
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homeostasis/metabolism
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Crygntm1a(EUCOMM)Wtsi mutation
(0 available);
any
Crygn mutation
(15 available)
Egr2tm2(cre)Pch mutation
(2 available);
any
Egr2 mutation
(31 available)
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nervous system
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N |
• mice exhibit no gross abnormalities in size or shape of all major nuclei of the superior olivary complex
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• reduced volume of the medial nucleus of the trapezoid body (MNTB) at P25 due to reduced neuron numbers without a reduction in cross sectional area
• small reduction in the lateral superior olive
• however, MNTB volume is normal at P4
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• in the medial nucleus of the trapezoid body
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hearing/vestibular/ear
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N |
• mice exhibit a non-significant improvement in ABR thresholds for click and noise burst stimuli and DPOAE growth functions, amplitudes and thresholds
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• amplitudes of wave IV are increased above 50 dB compared with controls
• however, overall latencies of ABR peaks are normal
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