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| Mice homozygous for the nose eyes ear mutation are runted, with proportionally smaller skeletons, shortened noses, shortened and domed skulls, reduced areal bone mineral density, diminished visceral and subcutaneous white adipose tissue, but not brown adipose tissue, and abnormal eyes and middle ears. The eyes have an enlarged anterior chamber, cloudy cornea, and severe peripheral anterior synechia of the irideocorneal angle. The middle ear shows inflammation as serous fluid with diffuse neutrophils with thickened epithelium. Elevated ABR thresholds are found in all homozygotes and homozygotes are infertile. | |||||||||||||||
- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Former Names B10.Cg H2h4-Sh3pxd2bnee/GrsrJ (Changed: 19-NOV-10 ) B10.A-H2h4/(4R)SgDvEgJ-Sh3pxd2bnee (Changed: 30-OCT-09 ) B10.Cg H2h4-nee/J (Changed: 28-OCT-09 ) B6.Cg H2h4-nee/J (Changed: 23-OCT-06 ) B6.Cg-H2h4-nee/J (Changed: 23-OCT-06 ) Type Coisogenic; Congenic; Major Histocompatibility Congenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Appearance
appearance: black with runted body size and shortened face
associated genotype: a/a Sh3pxd2bnee/Sh3pxd2bnee
appearance: black, normal size
associated genotype: a/a Sh3pxd2bnee/Sh3pxd2b+ or a/a Sh3pxd2b+/Sh3pxd2b+Description
Mice homozygous for the nose eyes ear mutation are runted, with proportionally smaller skeletons, shortened noses, shortened and domed skulls, reduced areal bone mineral density, diminished visceral and subcutaneous white adipose tissue, but not brown adipose tissue, and abnormal eyes and middle ears. The eyes have an enlarged anterior chamber, cloudy cornea, and severe peripheral anterior synechia of the irideocorneal angle. The middle ear shows inflammation as serous fluid with diffuse neutrophils with thickened epithelium. Elevated ABR thresholds are found in all homozygotes and homozygotes are infertile.Development
The nose eyes ear mutation arose spontaneously in the B10.A-H2h4/(4R)SgDvEgJ strain (stock #001150) at The Jackson Laboratory in 1999 when that strain was at generation N11F51. It was maintained by backcross-intercross mating to B10.A-H2h4/(4R)SgDvEgJ then by sibling breeding and in 2009 reached generation N3F7.
Control Information
| Control | ||
|---|---|---|
| +/? from the colony | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying H2h4 allele
001150 B10.A-H2h4/(4R)SgDvEgJ 004447 NOD.Cg-H2h4/DilTacUmmJ View Strains carrying H2h4 (2 strains)
Phenotype
Phenotype Information
Otitis Media, Susceptibility to - Models with phenotypic similarity to human disease where etiologies are distinct.2
2 Human genes are associated with this disease. Orthologs of those genes do not appear in the mouse genotype(s). assigned by genotype
Sh3pxd2bnee/Sh3pxd2bnee
B10.Cg-H2h4 Sh3pxd2bnee/GrsrJ
- growth/size phenotype
- abnormal postnatal growth
- mice stop growing at 2 months of age (MGI Ref ID J:153369)
- decreased body size (MGI Ref ID J:153369)
- craniofacial phenotype
- decreased cranium height (MGI Ref ID J:153369)
- domed cranium
- in all homozygotes (MGI Ref ID J:153369)
- short snout
- in all homozygotes (MGI Ref ID J:153369)
- skeleton phenotype
- abnormal skeleton development
- proportionally smaller skeleton (MGI Ref ID J:153369)
- decreased bone mineral density
- DEXA analysis shows reduced bone mineral density in both males and females with females having even lower density than males (MGI Ref ID J:153369)
- decreased cranium height (MGI Ref ID J:153369)
- domed cranium
- in all homozygotes (MGI Ref ID J:153369)
- vision/eye phenotype
- abnormal eye anterior chamber morphology
- the anterior chamber of the eye has a larger depth than normal (MGI Ref ID J:153369)
- enlarged eye anterior chamber (MGI Ref ID J:153369)
- corneal opacity
- eyes are typically bulging with white corneal opacities, which are present to varying degree in all homozygotes (MGI Ref ID J:153369)
- exophthalmos (MGI Ref ID J:153369)
- iris synechia
- severe peripheral anterior synechia are found in all homozygotes (MGI Ref ID J:153369)
- reproductive system phenotype
- infertility
- both female and male homozygotes are infertile (MGI Ref ID J:153369)
- hearing/vestibular/ear phenotype
- abnormal middle ear morphology
- serous fluid with diffuse neutrophils is found in the middle-ear cavities of all homozygotes and the surrounding epithelium is thickened by fibrous connective tissue and embedded neutrophils (MGI Ref ID J:153369)
- decreased brainstem auditory evoked potential
- elevated thresholds to broad-band click and 8, 16, and 32 kHz pure tone stimuli at all ages tested, from 34 to 92 days of age (MGI Ref ID J:153369)
- increased susceptibility to otitis media (MGI Ref ID J:153369)
- adipose tissue phenotype
- decreased white adipose tissue amount
- adult homozygotes have severely depleted visceral and subcutaneous white adipose tissue but normal brown adipose tissue, and this is less striking in young homozygotes (MGI Ref ID J:153369)
- immune system phenotype
- increased susceptibility to otitis media (MGI Ref ID J:153369)
This mouse can be used to support research in many areas including:Sh3pxd2bnee related
Developmental Biology Research
Craniofacial and Palate Defects
Eye Defects
Skeletal Defects
Endocrine Deficiency Research
Adipose Defects
Internal/Organ Research
Adipose Defects
Sensorineural Research
Eye Defects
Hearing Defects
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | H2h4 | ||
|---|---|---|---|
| Allele Name | h4 variant | ||
| Allele Type | Not Applicable | ||
| Common Name(s) | H-2h4; | ||
| Gene Symbol and Name | H2, histocompatibility-2, MHC | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | H-2; MHC-II; | ||
| General Note | This variant has been observed in the following strains: B10.A(4R) | ||
| Allele Symbol | Sh3pxd2bnee | ||
| Allele Name | nose eyes ear | ||
| Allele Type | Spontaneous | ||
| Strain of Origin | B10.A-H2h4/(4R)SgDvEgJ | ||
| Gene Symbol and Name | Sh3pxd2b, SH3 and PX domains 2B | ||
| Chromosome | 11 | ||
| Gene Common Name(s) | FAD49; FTHS; G431001E03Rik; HOFI; KIAA1295; RIKEN cDNA G431001E03 gene; TKS4; TSK4; | ||
| Molecular Note | A spontaneous mutation removes the A nucleotide at position 1303 (130delA). This mutation leads to a frame shift altering 37 amino acid before resulting in a premature stop codon and truncation of the protein product to 443 amino acids compared to the full-length 908 amino acids. The truncated protein lacks the fourth SH3 domain. [MGI Ref ID J:153369] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Sh3pxd2bnee, Pyrosequencing
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Selected Reference(s)
Mao M; Thedens DR; Chang B; Harris BS; Zheng QY; Johnson KR; Donahue LR; Anderson MG. 2009. The podosomal-adaptor protein SH3PXD2B is essential for normal postnatal development. Mamm Genome 20(8):462-75. [PubMed: 19669234] [MGI Ref ID J:153369]
Additional References
H2h4 relatedSh3pxd2bnee relatedBraley-Mullen H; Sharp GC; Medling B; Tang H. 1999. Spontaneous autoimmune thyroiditis in NOD.H-2h4 mice. J Autoimmun 12(3):157-65. [PubMed: 10222025] [MGI Ref ID J:54563]
Braley-Mullen H; Yu S. 2000. Early requirement for B cells for development of spontaneous autoimmune thyroiditis in NOD.H-2h4 mice J Immunol 165(12):7262-9. [PubMed: 11120860] [MGI Ref ID J:66106]
Fang Y; Sharp GC; Braley-Mullen H. 2008. Interleukin-10 promotes resolution of granulomatous experimental autoimmune thyroiditis. Am J Pathol 172(6):1591-602. [PubMed: 18467701] [MGI Ref ID J:136188]
Horie I; Abiru N; Nagayama Y; Kuriya G; Saitoh O; Ichikawa T; Iwakura Y; Eguchi K. 2009. T helper type 17 immune response plays an indispensable role for development of iodine-induced autoimmune thyroiditis in nonobese diabetic-H2h4 mice. Endocrinology 150(11):5135-42. [PubMed: 19797122] [MGI Ref ID J:158234]
Hutchings PR; Verma S; Phillips JM; Harach SZ; Howlett S; Cooke A. 1999. Both CD4(+) T cells and CD8(+) T cells are required for iodine accelerated thyroiditis in NOD mice. Cell Immunol 192(2):113-21. [PubMed: 10087179] [MGI Ref ID J:54320]
Levisetti MG; Lewis DM; Suri A; Unanue ER. 2008. Weak proinsulin peptide-major histocompatibility complexes are targeted in autoimmune diabetes in mice. Diabetes 57(7):1852-60. [PubMed: 18398138] [MGI Ref ID J:138230]
Miyashita N; Migita S; Moriwaki K. 1987. Effects of H-2 complex and non-H-2 background on urethane-induced chromosomal aberrations in mice. Mutat Res 176(1):59-67. [PubMed: 3099189] [MGI Ref ID J:109945]
Nagayama Y; Horie I; Saitoh O; Nakahara M; Abiru N. 2007. CD4(+)CD25(+) naturally occurring regulatory T cells and not lymphopenia play a role in the pathogenesis of iodide-induced autoimmune thyroiditis in NOD-H2(h4) mice. J Autoimmun 29(2-3):195-202. [PubMed: 17826032] [MGI Ref ID J:125178]
Podolin PL; Pressey A; DeLarato NH; Fischer PA; Peterson LB; Wicker LS. 1993. I-E+ nonobese diabetic mice develop insulitis and diabetes. J Exp Med 178(3):793-803. [PubMed: 8350054] [MGI Ref ID J:14178]
Sharma R; Traore K; Trush MA; Rose NR; Burek CL. 2008. Intracellular adhesion molecule-1 up-regulation on thyrocytes by iodine of non-obese diabetic.H2(h4) mice is reactive oxygen species-dependent. Clin Exp Immunol 152(1):13-20. [PubMed: 18241232] [MGI Ref ID J:133583]
Yu S; Sharp GC; Braley-Mullen H. 2008. TGF-beta promotes thyroid epithelial cell hyperplasia and fibrosis in IFN-gamma-deficient NOD.H-2h4 mice. J Immunol 181(3):2238-45. [PubMed: 18641364] [MGI Ref ID J:139224]
Yang B; Tian C; Zhang ZG; Han FC; Azem R; Yu H; Zheng Y; Jin G; Arnold JE; Zheng QY. 2011. Sh3pxd2b mice are a model for craniofacial dysmorphology and otitis media. PLoS One 6(7):e22622. [PubMed: 21818352] [MGI Ref ID J:175853]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, RG10/RG30.
Purchasing information
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $1980.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
- Cryorecovery of Strains Needing Progeny Testing.
At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks.Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation. Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 (from U.S.A., Canada and Puerto Rico only) or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
| Pricing for International shipping destinations |
|
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2574.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
- Cryorecovery of Strains Needing Progeny Testing.
At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks.Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation. Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 (from U.S.A., Canada and Puerto Rico only) or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
General Supply Notes
- This strain is included in the Mouse Mutant Resource collection.
- Genomic DNA is available for this strain from the Mouse DNA Resource.
Control Information
| Control | ||
|---|---|---|
| +/? from the colony | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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 Information
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