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- 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
Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Generation ?+N1p (05-FEB-06)
Generation DefinitionsDescription
This targeted mutant was created and characterized by Deltagen, Inc. View phenotypic data developed by Deltagen.Development
The construct insert is reported to be "SA-IRES-lacZ-Neo555G/Kan." Upon arrival at The Jackson Laboratory, these mice had been backcrossed at least 6 generations to C57BL/6 mice.
Related Strains
lacZ Expression Strains
View lacZ Expression Strains (245 strains)
Additional Web Information
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.
Efemp2tm1Dgen/Efemp2+
involves: 129P2/OlaHsd * C57BL/6
- cardiovascular system phenotype
- abnormal aorta tunica media morphology
- aortas of heterozygotes housed in standard cages exhibit localized regions of disorganized extracellular matrix and collagen fibers between 10-15% of the medial smooth muscle cell junctions (MGI Ref ID J:129341)
- heterozygotes housed in enriched cages show fewer disorganized regions in the aorta than in standard cages (MGI Ref ID J:129341)
- normal phenotype
- no abnormal phenotype detected
- no significant differences in physical examinations, necropsy including body length, body weight, and organ weight measurements, histological examination of tissues and organs, bone marrow section evaluations, complete blood counts and differentials, clinical chemistry panels, densitometry, behavior tests, fertility, or aging studies detected in the heterozygous mutant mice when compared with age- and gender-matched wild-type control mice (MGI Ref ID J:101679)
Efemp2tm1Dgen/Efemp2tm1Dgen
involves: 129P2/OlaHsd * C57BL/6
- mortality/aging
- complete embryonic lethality during organogenesis
- homozygous embryos die around E12.5, with embryos appearing partially abnormal and/or partially resorbed by E12.5 (MGI Ref ID J:101679)
- complete perinatal lethality
- cardiovascular system phenotype
- abnormal artery morphology
- abnormal aorta morphology
- at E15.5 the aorta is noticeably tortuous and irregular and this becomes more severe with age (MGI Ref ID J:106902)
- abnormal aorta wall morphology
- at E12.5 the outer diameter is reduced to 1/2 to 2/3 of wild-type; however, the number of cells in the wall is not increased compared to wild-type (MGI Ref ID J:106902)
- abnormal aorta elastic tissue morphology
- no continuous elatic lamina is seen at any age from E12.5 to P1 (MGI Ref ID J:106902)
- at E14.5 irregular elastin aggregates are seen and these become larger and more numerous with age (MGI Ref ID J:106902)
- at P1 decreased desmosine levels indicate a 94% decrease in elastin cross linking (MGI Ref ID J:106902)
- the aortic elastic laminae is complete disarranged unlike in wild-type mice (MGI Ref ID J:154659)
- the elastogenesis is abolished unlike in wild-type mice (MGI Ref ID J:154659)
- abnormal aorta smooth muscle morphology
- at E12.5 aortic smooth muscle cells appear rounder and less stretched although this becomes less obvious at E13.5 and later ages (MGI Ref ID J:106902)
- increased aorta wall thickness
- the walls are about twice as thick as in wild-type mice (MGI Ref ID J:106902)
- descending aorta stenosis
- uniform narrowing (stenosis) of the descending aorta in mutant embryos at embryonic day 12.5 (E12.5) (MGI Ref ID J:106902)
- artery stenosis (MGI Ref ID J:106902)
- descending aorta stenosis
- uniform narrowing (stenosis) of the descending aorta in mutant embryos at embryonic day 12.5 (E12.5) (MGI Ref ID J:106902)
- aneurysm
- arterial aneurysms (MGI Ref ID J:154659)
- hemorrhage
- resulting from ruptured arteries (MGI Ref ID J:106902)
- respiratory system phenotype
- abnormal pulmonary elastic fiber morphology
- at P1 no fine elastic fibers are detected and decreased desmosine levels indicate an 88% decrease in elastin cross linking (MGI Ref ID J:106902)
- emphysema
- over expanded with markedly enlarged distal airspaces (MGI Ref ID J:106902)
- muscle phenotype
- abnormal aorta smooth muscle morphology
- at E12.5 aortic smooth muscle cells appear rounder and less stretched although this becomes less obvious at E13.5 and later ages (MGI Ref ID J:106902)
- integument phenotype
- abnormal hypodermis morphology
- at P1 no fine elastic fibers are detected in the hypodermal connective tissue; however, no gross changes in skin condition are seen (MGI Ref ID J:106902)
This mouse can be used to support research in many areas including:
Developmental Biology Research
Research Tools
lacZ Expression
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Efemp2tm1Dgen | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Deltagen | ||
| Allele Type | Targeted (Reporter) | ||
| Common Name(s) | Fbln4-; fibulin-4-; | ||
| Strain of Origin | 129P2/OlaHsd | ||
| ES Cell Line Name | E14 | ||
| ES Cell Line Strain | 129P2/OlaHsd | ||
| Site of Expression | lacZ is detected in most tissues examined: brain, spinal cord, eyes, Harderian glands, thymus, spleen, lymph nodes, aorta, heart, lung, liver, gallbladder, pancreas, kidney, urinary bladder, trachea, larynx, esophagus, thyroid gland, pituitary gland, adrenal glands, salivary glands, tongue, skeletal muscle, skin, male and female reproductive systems. | ||
| Expressed Gene | lacZ, beta-galactosidase, E. coli | ||
| Molecular Note | A bacterial lacZ gene was inserted into the gene such that the endogenous gene promoter drives expression of beta-galactosidase. While RT-PCR analysis detects a gene transcript in all tissues tested, strong beta-galactosidase expression from this alleleis detectable in brain, spinal cord, eyes, Harderian glands, thymus, spleen, lymph nodes, aorta, heart, lung, liver, gallbladder, pancreas, kidney, urinary bladder, trachea, larynx, esophagus, thyroid gland, pituitary gland, adrenal glands, salivary glands, tongue, skeletal muscle, skin, male and female reproductive systems. In many tissues lacZ is expressed in smooth muscle cells. | ||
| Gene Symbol and Name | Efemp2, epidermal growth factor-containing fibulin-like extracellular matrix protein 2 | ||
| Chromosome | 19 | ||
| Gene Common Name(s) | 0610011K11Rik; ARCL1B; FBLN4; MBP1; RIKEN cDNA 0610011K11 gene; UPH1; fibulin 4; fibulin-4; | ||
Genotyping
Genotyping Information
Genotyping Protocols
Efemp2tm1Dgen, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Additional References
Efemp2tm1Dgen relatedCudilo E; Al Naemi H; Marmorstein L; Baldwin AL. 2007. Knockout mice: is it just genetics? Effect of enriched housing on fibulin-4(+/-) mice. PLoS ONE 2(2):e229. [PubMed: 17311093] [MGI Ref ID J:129341]
Deltagen Inc. 2005. NIH initiative supporting placement of Deltagen, Inc. mice into public repositories MGI Direct Data Submission :. [MGI Ref ID J:101679]
Horiguchi M; Inoue T; Ohbayashi T; Hirai M; Noda K; Marmorstein LY; Yabe D; Takagi K; Akama TO; Kita T; Kimura T; Nakamura T. 2009. Fibulin-4 conducts proper elastogenesis via interaction with cross-linking enzyme lysyl oxidase. Proc Natl Acad Sci U S A 106(45):19029-34. [PubMed: 19855011] [MGI Ref ID J:154659]
Huang J; Davis EC; Chapman SL; Budatha M; Marmorstein LY; Word RA; Yanagisawa H. 2010. Fibulin-4 deficiency results in ascending aortic aneurysms: a potential link between abnormal smooth muscle cell phenotype and aneurysm progression. Circ Res 106(3):583-92. [PubMed: 20019329] [MGI Ref ID J:170883]
McLaughlin PJ; Chen Q; Horiguchi M; Starcher BC; Stanton JB; Broekelmann TJ; Marmorstein AD; McKay B; Mecham R; Nakamura T; Marmorstein LY. 2006. Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice. Mol Cell Biol 26(5):1700-9. [PubMed: 16478991] [MGI Ref ID J:106902]
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 - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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 - Standard.
We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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 Induced Mutant Resource Colony collection.
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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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