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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Atoh1tm2Hzo
targeted mutation 2, Huda Y Zoghbi
MGI:2155983
Summary 6 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Atoh1tm2Hzo/Atoh1tm2Hzo Not Specified MGI:2183424
ht2
 		Atoh1tm2Hzo/Atoh1tm3.1Hzo involves: 129S7/SvEvBrd * C57BL/6J * FVB/N * SJL MGI:3767182
cn3
 		Atoh1tm2Hzo/Atoh1tm3Hzo
Tg(Fabp1-cre)1Jig/0 		involves: 129S7/SvEvBrd * C57BL/6J * FVB/N * SJL MGI:3767181
cn4
 		Atoh1tm2Hzo/Atoh1tm3Hzo
Tg(Vil1-cre)997Gum/0 		involves: 129S7/SvEvBrd * C57BL/6J * SJL MGI:3767180
cx5
 		Atoh1tm2Hzo/Atoh1+
Tcf4tm1Zhu/Tcf4+ 		involves: 129P2/OlaHsd MGI:3776856
cx6
 		Atoh1tm2Hzo/Atoh1+
Gfi1tm1Sho/Gfi1tm1Sho 		involves: 129S1/Sv * C57BL/6J MGI:3696821


Genotype
MGI:2183424
hm1
Allelic
Composition		 Atoh1tm2Hzo/Atoh1tm2Hzo
Genetic
Background		 Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
neonatal lethality, complete penetrance ( J:60393 , J:155760 )
• animals die shortly after birth (J:60393)
• reported to make occasional gasps after birth (J:155760)
• unresponsive by 30-45 minutes after birth (J:155760)

hearing/vestibular/ear
absent vestibular hair cells ( J:55694 )
• hair cells were absent from utricles and cochlea

homeostasis/metabolism

respiratory system
decreased pulmonary respiratory rate ( J:155760 )
• diaphragmatic electromyograms indicate slow and variable respiratory rhythms involving the phrenic nerve
• rhythms also slow when recorded from hypoglossal and C4 nerves when the diaphragm is not part of the experimental preparation
• glutamatergic modulators such as dihydrokainic acid and ampakine (CX546) restore near normal rhythm and pattern in expermimental preparations
• substance P increases respiratory rhythm but does not restore normal frequency or pattern
• norepinephrin establishes a similar slow, regular pace in both mutant and control experimental preparations
respiratory failure ( J:60393 )
• animals do not breathe after birth

nervous system
nervous system phenotype ( J:60393 )
N
• normal hindbrain: normal nucleus tractus solitarus, nucleus ambiguus, dorsal vagus nucleus and trigeminal ganglion
absent vestibular hair cells ( J:55694 )
• hair cells were absent from utricles and cochlea
abnormal hindbrain development ( J:105186 )
• in null embryos, certain nuclei of the rostrolateral region of the hindbrain that originate in the cerebellar rhombic lip fail to form; nuclei such as the pontine nuclei and the external granule layer do not seem to form in the rostrolateral hindbrain but are present at more caudal positions in mutant brains
abnormal cerebellum external granule cell layer morphology ( J:60393 )
• absent external granule layer: migrating granule cells absent as early as E14 in rhombic lip
abnormal cerebellar Purkinje cell layer ( J:60393 )
• disorganized and cells localized at periphery of cerebellum




Genotype
MGI:3767182
ht2
Allelic
Composition		 Atoh1tm2Hzo/Atoh1tm3.1Hzo
Genetic
Background		 involves: 129S7/SvEvBrd * C57BL/6J * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
Atoh1tm3.1Hzo mutation (0 available); any Atoh1 mutation (37 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
neonatal lethality, complete penetrance ( J:128312 )
• die at birth and are stated to be phenotypically indistinguishable from Atoh1tm2Hzo homozygous mice




Genotype
MGI:3767181
cn3
Allelic
Composition		 Atoh1tm2Hzo/Atoh1tm3Hzo
Tg(Fabp1-cre)1Jig/0
Genetic
Background		 involves: 129S7/SvEvBrd * C57BL/6J * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
Atoh1tm3Hzo mutation (1 available); any Atoh1 mutation (37 available)
Tg(Fabp1-cre)1Jig mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
mortality/aging ( J:128312 )
N
• unlike mice with complete recombination of the floxed allele in the entire intestinal epithelium, mice with a mosaic pattern of recombination show no increase in mortality up to at least 1 year of age

digestive/alimentary system
abnormal small intestine crypts of Lieberkuhn morphology ( J:128312 )
• the small intestine contains patches of abnormal crypts with a recombined allele mixed with areas of normal crypts with an unrecombined allele
• crypts with the recombined allele completely lack goblet, Paneth, and enteroendocrine cells instead containing only absorptive enterocytes
• a significant increase in BrdU+ cells and mitotic figures is seen in crypts with a recombined allele
• crypts with a recombined allele are smaller than neighboring crypts in which recombination did not occur
impaired intestine regeneration ( J:128312 )
• the adaptive response following resection of the middle 50% of the small bowel is blunted with a smaller increase in crypt depth compared to control mice

endocrine/exocrine glands
abnormal small intestine crypts of Lieberkuhn morphology ( J:128312 )
• the small intestine contains patches of abnormal crypts with a recombined allele mixed with areas of normal crypts with an unrecombined allele
• crypts with the recombined allele completely lack goblet, Paneth, and enteroendocrine cells instead containing only absorptive enterocytes
• a significant increase in BrdU+ cells and mitotic figures is seen in crypts with a recombined allele
• crypts with a recombined allele are smaller than neighboring crypts in which recombination did not occur




Genotype
MGI:3767180
cn4
Allelic
Composition		 Atoh1tm2Hzo/Atoh1tm3Hzo
Tg(Vil1-cre)997Gum/0
Genetic
Background		 involves: 129S7/SvEvBrd * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
Atoh1tm3Hzo mutation (1 available); any Atoh1 mutation (37 available)
Tg(Vil1-cre)997Gum mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
postnatal lethality, complete penetrance ( J:128312 )
• no mutants are found at P14




Genotype
MGI:3776856
cx5
Allelic
Composition		 Atoh1tm2Hzo/Atoh1+
Tcf4tm1Zhu/Tcf4+
Genetic
Background		 involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
Tcf4tm1Zhu mutation (0 available); any Tcf4 mutation (58 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
neonatal lethality ( J:125313 )
• mice die within the first 24 hours of birth but no embryonic lethality is observed as previously noted

nervous system
abnormal brain development ( J:125313 )
• the pontine nuclei is disrupted




Genotype
MGI:3696821
cx6
Allelic
Composition		 Atoh1tm2Hzo/Atoh1+
Gfi1tm1Sho/Gfi1tm1Sho
Genetic
Background		 involves: 129S1/Sv * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atoh1tm2Hzo mutation (1 available); any Atoh1 mutation (37 available)
Gfi1tm1Sho mutation (0 available); any Gfi1 mutation (31 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
abnormal cochlear hair cell morphology ( J:80025 )
• at E15.5, the characteristic rows of IHCs and OHCs are not as clearly defined in the basal cochlea
• at E17.5, mutants display disorganization of hair cells and decreased hair cell numbers in the basal cochlea
• at P0, the apical cochlea shows little to no hair cell loss, but exhibits a disorganization similar to that seen in basal cochlea at E15.5
cochlear hair cell degeneration ( J:80025 )
• cochlear IHCs and OHCs are initially specified by E15.5 but are subsequently lost in a basal-to-apical gradient at P0
• by P3, the basal cochlea has few hair cells while the medial cochlea still retains most IHCs but has lost almost all OHCs
• in all cases, OHCs in a given region degenerate prior to IHCs cells
abnormal vestibular hair cell morphology ( J:80025 )
• at P0, the saccule shows disorganization of the hair cell layer, with some hair cells found in the supporting cell layer
• in contrast, cristae exhibit a relatively normal development of hair cells
abnormal vestibular hair cell stereociliary bundle morphology ( J:80025 )
• at P0, mutant vestibular hair cells appear to have less well organized stereocillia relative to wild-type hair cells

nervous system
abnormal cochlear hair cell morphology ( J:80025 )
• at E15.5, the characteristic rows of IHCs and OHCs are not as clearly defined in the basal cochlea
• at E17.5, mutants display disorganization of hair cells and decreased hair cell numbers in the basal cochlea
• at P0, the apical cochlea shows little to no hair cell loss, but exhibits a disorganization similar to that seen in basal cochlea at E15.5
cochlear hair cell degeneration ( J:80025 )
• cochlear IHCs and OHCs are initially specified by E15.5 but are subsequently lost in a basal-to-apical gradient at P0
• by P3, the basal cochlea has few hair cells while the medial cochlea still retains most IHCs but has lost almost all OHCs
• in all cases, OHCs in a given region degenerate prior to IHCs cells
abnormal vestibular hair cell morphology ( J:80025 )
• at P0, the saccule shows disorganization of the hair cell layer, with some hair cells found in the supporting cell layer
• in contrast, cristae exhibit a relatively normal development of hair cells
abnormal vestibular hair cell stereociliary bundle morphology ( J:80025 )
• at P0, mutant vestibular hair cells appear to have less well organized stereocillia relative to wild-type hair cells




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11/12/2024
MGI 6.24 		The Jackson Laboratory