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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
Strain Information
Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered Mutant Mice. Mating System +/+ sibling x Heterozygote (Female x Male) Species laboratory mouse Donating Investigator Huda Zoghbi, Baylor College of Medicine Description
Mice that are heterozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Homozygous mutant mice have a perinatal lethal phenotype and die shortly after birth. No gene product (protein) is detected in resting chondrocytes by immunohistochemical analysis of embryonic age 18.5 homozygotes. Beta-galactosidase X-gal staining of neural tissue from embryonic day 14.5 and newborn (postnatal day 0) aged homozygous and heterozygous mice mimicks the endogenous expression pattern. Mice homozygous for this mutation exhibit a phenotype similar to the phenotype observed in mice homozygous for the null (loss of function) targeted mutation. Homozygotes lack cerebellar granule neurons, cochlear and ventricular hair cells, and the pontine nuclei in the brain stem. This mutant mouse strain may be useful in studies of brain and inner ear development.Development
A targeting vector containing lacZ, neomycin resistance and herpes simplex virus thymidine kinase genes was used to disrupt the entire coding region. The construct was electroporated into 129S7/SvEvBrd-Hprtb-m2derived AB2.2 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. The resulting chimeric animals were crossed to C57BL/6 mice, and then backcrossed to the same for 10 generations.
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
lacZ Expression Strains
View lacZ Expression Strains (176 strains)
007684 STOCK Tg(Atoh1-cre/ESR1)14Fsh/J
Additional Web Information
Congenic Nomenclature
Fluorescent Proteins/lacZ Systems
Phenotype
Phenotype Information
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Atoh1tm2Hzo/Atoh1tm2Hzo
Background Not Specified
- lethality-prenatal/perinatal
- neonatal lethality (MGI Ref ID J:60393)
- animals die shortly after birth
- hearing/vestibular/ear phenotype
- absent cochlear hair cells (MGI Ref ID J:55694)
- absent vestibular hair cells (MGI Ref ID J:55694)
- hair cells were absent from utricles and cochlea
- homeostasis/metabolism phenotype
- cyanosis (MGI Ref ID J:60393)
- respiratory system phenotype
- respiratory failure (MGI Ref ID J:60393)
- animals do not breathe after birth
- nervous system phenotype
- *normal* nervous system phenotype (MGI Ref ID J:60393)
- normal hindbrain: normal nucleus tractus solitarus, nucleus ambiguus, dorsal vagus nucleus and trigeminal ganglion
- abnormal cerebellar Purkinje cell layer (MGI Ref ID J:60393)
- disorganized and cells localized at periphery of cerebellum
- abnormal external granule cell layer morphology (MGI Ref ID J:60393)
- absent external granule layer: migrating granule cells absent as early as E14 in rhombic lip
- abnormal hindbrain development (MGI Ref ID 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
- absent cerebellar foliation (MGI Ref ID J:60393)
- absent cochlear hair cells (MGI Ref ID J:55694)
- absent vestibular hair cells (MGI Ref ID J:55694)
- hair cells were absent from utricles and cochlea
- small cerebellum (MGI Ref ID J:60393)
This mouse can be used to support research in many areas including:
Developmental Biology Research
Neurodevelopmental Defects
Perinatal Lethality (Homozygous)
Neurobiology Research
lacZ expression in neural tissue
Cerebellar Defects
Neurodevelopmental Defects
Vestibular and Hearing Defects
Research Tools
lacZ Expression
Neurobiology Research (cell marker)
Sensorineural Research
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Atoh1tm2Hzo | ||
|---|---|---|---|
| Allele Name | targeted mutation 2, Huda Y Zoghbi | ||
| Allele Type | Targeted (Reporter) | ||
| Common Name(s) | Math1/lacZ; Math1-; Math1beta-Gal; Math1lacZ; | ||
| Mutation Made By | Huda Zoghbi, Baylor College of Medicine | ||
| Site of Expression | lacZ replaces/mimics endogenous gene expression | ||
| Gene Symbol and Name | Atoh1, atonal homolog 1 (Drosophila) | ||
| Chromosome | 6 | ||
| Gene Common Name(s) | ATH1; HATH1; MATH-1; Math1; RGD1565171; bHLHa14; | ||
| Molecular Note | A lacZ-PGK-neomycin resistance cassette replaced the coding region and placed lacZ under control of the endogenous promoter. [MGI Ref ID J:60393] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Atohtm2Hzo), SEP PCR, vers. 1
Helpful Links
Optimizing PCR Protocols
References
References
Selected Reference(s)
Ben-Arie N; Hassan BA; Bermingham NA; Malicki DM; Armstrong D; Matzuk M; Bellen HJ; Zoghbi HY. 2000. Functional conservation of atonal and Math1 in the CNS and PNS. Development 127(5):1039-48. [PubMed: 10662643] [MGI Ref ID J:60393]
Additional References
Atoh1tm2Hzo relatedBermingham NA; Hassan BA; Price SD; Vollrath MA; Ben-Arie N; Eatock RA; Bellen HJ; Lysakowski A; Zoghbi HY. 1999. Math1: an essential gene for the generation of inner ear hair cells. Science 284(5421):1837-41. [PubMed: 10364557] [MGI Ref ID J:55694]
Bermingham NA; Hassan BA; Wang VY; Fernandez M; Banfi S; Bellen HJ; Fritzsch B; Zoghbi HY. 2001. Proprioceptor pathway development is dependent on math1. Neuron 30(2):411-22. [PubMed: 11395003] [MGI Ref ID J:69624]
Chizhikov VV; Lindgren AG; Currle DS; Rose MF; Monuki ES; Millen KJ. 2006. The roof plate regulates cerebellar cell-type specification and proliferation. Development 133(15):2793-804. [PubMed: 16790481] [MGI Ref ID J:119033]
Driver EC; Pryor SP; Hill P; Turner J; Ruther U; Biesecker LG; Griffith AJ; Kelley MW. 2008. Hedgehog signaling regulates sensory cell formation and auditory function in mice and humans. J Neurosci 28(29):7350-8. [PubMed: 18632939] [MGI Ref ID J:139256]
Englund C; Kowalczyk T; Daza RA; Dagan A; Lau C; Rose MF; Hevner RF. 2006. Unipolar brush cells of the cerebellum are produced in the rhombic lip and migrate through developing white matter. J Neurosci 26(36):9184-95. [PubMed: 16957075] [MGI Ref ID J:112207]
Flora A; Garcia JJ; Thaller C; Zoghbi HY. 2007. The E-protein Tcf4 interacts with Math1 to regulate differentiation of a specific subset of neuronal progenitors. Proc Natl Acad Sci U S A 104(39):15382-7. [PubMed: 17878293] [MGI Ref ID J:125313]
Fritzsch B; Matei VA; Nichols DH; Bermingham N; Jones K; Beisel KW; Wang VY. 2005. Atoh1 null mice show directed afferent fiber growth to undifferentiated ear sensory epithelia followed by incomplete fiber retention. Dev Dyn 233(2):570-83. [PubMed: 15844198] [MGI Ref ID J:98393]
Jensen P; Smeyne R; Goldowitz D. 2004. Analysis of cerebellar development in math1 null embryos and chimeras. J Neurosci 24(9):2202-11. [PubMed: 14999071] [MGI Ref ID J:90130]
Jensen P; Zoghbi HY; Goldowitz D. 2002. Dissection of the cellular and molecular events that position cerebellar Purkinje cells: a study of the math1 null-mutant mouse. J Neurosci 22(18):8110-6. [PubMed: 12223565] [MGI Ref ID J:79141]
Kiernan AE; Pelling AL; Leung KK; Tang AS; Bell DM; Tease C; Lovell-Badge R; Steel KP; Cheah KS. 2005. Sox2 is required for sensory organ development in the mammalian inner ear. Nature 434(7036):1031-5. [PubMed: 15846349] [MGI Ref ID J:98458]
Krizhanovsky V; Ben-Arie N. 2006. A novel role for the choroid plexus in BMP-mediated inhibition of differentiation of cerebellar neural progenitors. Mech Dev 123(1):67-75. [PubMed: 16325379] [MGI Ref ID J:104282]
Li S; Mark S; Radde-Gallwitz K; Schlisner R; Chin MT; Chen P. 2008. Hey2 functions in parallel with Hes1 and Hes5 for mammalian auditory sensory organ development. BMC Dev Biol 8:20. [PubMed: 18302773] [MGI Ref ID J:135642]
Machold R; Fishell G. 2005. Math1 is expressed in temporally discrete pools of cerebellar rhombic-lip neural progenitors. Neuron 48(1):17-24. [PubMed: 16202705] [MGI Ref ID J:105186]
Matei V; Pauley S; Kaing S; Rowitch D; Beisel KW; Morris K; Feng F; Jones K; Lee J; Fritzsch B. 2005. Smaller inner ear sensory epithelia in Neurog 1 null mice are related to earlier hair cell cycle exit. Dev Dyn 234(3):633-50. [PubMed: 16145671] [MGI Ref ID J:102293]
Shroyer NF; Helmrath MA; Wang VY; Antalffy B; Henning SJ; Zoghbi HY. 2007. Intestine-specific ablation of mouse atonal homolog 1 (Math1) reveals a role in cellular homeostasis. Gastroenterology 132(7):2478-88. [PubMed: 17570220] [MGI Ref ID J:128312]
Shroyer NF; Wallis D; Venken KJ; Bellen HJ; Zoghbi HY. 2005. Gfi1 functions downstream of Math1 to control intestinal secretory cell subtype allocation and differentiation. Genes Dev 19(20):2412-7. [PubMed: 16230531] [MGI Ref ID J:102106]
Swanson DJ; Tong Y; Goldowitz D. 2005. Disruption of cerebellar granule cell development in the Pax6 mutant, Sey mouse. Brain Res Dev Brain Res 160(2):176-93. [PubMed: 16289327] [MGI Ref ID J:103551]
Wallis D; Hamblen M; Zhou Y; Venken KJ; Schumacher A; Grimes HL; Zoghbi HY; Orkin SH; Bellen HJ. 2003. The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival. Development 130(1):221-32. [PubMed: 12441305] [MGI Ref ID J:80025]
Wang VY; Hassan BA; Bellen HJ; Zoghbi HY. 2002. Drosophila atonal fully rescues the phenotype of Math1 null mice: new functions evolve in new cellular contexts. Curr Biol 12(18):1611-6. [PubMed: 12372255] [MGI Ref ID J:79026]
Wang VY; Rose MF; Zoghbi HY. 2005. Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum. Neuron 48(1):31-43. [PubMed: 16202707] [MGI Ref ID J:105185]
Woods C; Montcouquiol M; Kelley MW. 2004. Math1 regulates development of the sensory epithelium in the mammalian cochlea. Nat Neurosci 7(12):1310-8. [PubMed: 15543141] [MGI Ref ID J:95110]
Yang Q; Bermingham NA; Finegold MJ; Zoghbi HY. 2001. Requirement of Math1 for secretory cell lineage commitment in the mouse intestine. Science 294(5549):2155-8. [PubMed: 11739954] [MGI Ref ID J:73284]
Health & husbandry
Health & Colony Maintenance Information
Colony Maintenance
Breeding & Husbandry When maintaining a live colony, these mice are bred as heterozygotes due to the homozygous perinatal lethal phenotype. Mating System +/+ sibling x Heterozygote (Female x Male)
Purchasing information
Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $1900.00
Additional Supply Details
| Pricing for International shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $2470.00
Additional Supply Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
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Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
General Terms and Conditions
See Terms of Use
The Jackson Laboratory's Genotype Promise
The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.Ordering and Purchasing Information
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For Licensing and Use Restrictions view the link(s) below:
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Contact information
General inquiries
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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