Search Criteria: Research Area is "Cell Biology Research: Defects in Extracellular Matrix Molecules"
New Strains Awaiting Transfer from the Donor
Additional Register Interest Strains
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 016917 | B6.129P2-Lamc1tm1Strl/J | Repository- Live |
| These fLamγ1 mice possess a frt-flanked neomycin resistance (neo) cassette upstream of exon 2, and loxP sites flanking exon 2 of the laminin, gamma 1 (Lamc1) targeted gene. Homozygotes are viable, fertile, and normal in size. The donating investigator reports that the frt-flanked neo cassette that is present upstream of the floxed exon and does not appear to effect expression of Lamc1. Laminins are extracellular matrix proteins composed of heterotrimeric α, β, and γ chains. Laminins regulate basement membrane assembly, and cell proliferation, differentiation, viability, and function. When these mutant mice are bred to mice that express Cre recombinase, resulting offspring will have exon 2 deleted in the cre-expressing tissue. For example, when Lamininγ1 expression is disrupted in Schwann cells (Tg(Mpz-cre)1Mfel; see Stock No. 017927 for example), th ..... For more information please see the full phenotype on the strain data sheet | ||
| 006141 | B6.129S2-Thbs1tm1Hyn/J | Repository- Live |
| Mice homozygous for this targeted mutation are viable and fertile, with an approximate 20% decrease in embryo/neonate viability and a mild and variable lordotic curvature of the spine apparent from birth. Homozygous mice have an abnormal, but no full length transcript in multiple tissues. Western analysis confirmed the absence of the protein in platelets. Homozygotes exhibit an increase in the number of circulating white blood cells. During the first four to ten weeks of life, homozygotes exhibit patches of acute and organizing pneumonia. At later time points, there is considerable hyperplasia of the various epithelial cell lineages. Mutant mice also have an increased number of retinal endothelial cells and inappropriate remodeling and maturation of retinal vasculature following injury. On the FVB/N background, spontaneous tumor growth and vasculature are significantly increased compared to wildtype. Mutant mice may be useful in studies of inflammatory responses in the lungs, eye, and ..... For more information please see the full phenotype on the strain data sheet | ||
| 016221 | B6;129-Ip6k2tm1Dlin/J | Repository- Live |
| In this strain, a floxed-neo cassette replaces the first 202 nucleotides of exon 2 of the inositol hexaphosphate kinase 2 (Ip6k2) gene, abolishing gene expression. Homozygotes are viable, fertile, and normal in size. IP6K2 suppresses growth and increases apoptosis during cell stress by catalyzing the synthesis of inositol polyphosphates. These compounds are involved in the regulation of telomere length, protein phosphorylation, and cell growth and movement. After chronic exposure to the carcinogen 4-nitroquinoline 1-oxide (4-NQO) these mice exhibit a four-fold increase in the incidence of invasive squamous cell carcinoma (SCC) formation in the tongue, oral cavity, and esophagus. They also display relative resistance to ionizing radiation and exhibit increased survival following total body irradiation. Cells from these mice are relatively resistant to growth-suppression by interferon-β (IFN-β). These mice may be useful for studying the mechanisms of IP6K2-dependent ..... For more information please see the full phenotype on the strain data sheet | ||
| 000251 | AEJ.Cg-ae +/a Gdf5bp-H/J | Cryopreserved - Ready for recovery |
| 002995 | B6 x C.B10-H2b/LiMcdJ-Fbn2fp-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the recessive fused phalanges 2 Jackson mutation (Fbn2fp-2J) have syndactyly of the second, third, and/or the fourth digits of the hindlimbs with <20% showing involvement of all three digits. This syndactyly likely results from defective mesenchyme differentiation rather than failed interdigital apoptosis (Arteaga-Solis et al., 2001). This mutant has a less severe phenotype than other Fbn2 mutants do, and shows no involvement of the digits of the forelimbs. Whether this is due to the allele or the genetic background has not been determined. (Chaudhry et al., 2001.) | ||
| 007248 | B6.129(FVB)-Col1a2tm1.1Mcbr/J | Cryopreserved - Ready for recovery |
| These mice harbor a point mutation knock-in patterned after the variant found among Old Order Amish kindred (OOA) of Lancaster County, Pennsylvania. Mice exhibit reduced body mass and reduced bone mass density by 2 months of age. Homozygotes do not survive to weaning age. These mutant mice express mutant type I collagen similar to that observed in humans with osteogenesis imperfecta. Incorporation of the point mutation was verified by sequence analysis. Mutant mRNA is detected by RT-PCR analysis of total RNA. | ||
| 002274 | B6.129S-Itga5tm1Hyn/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Itga5tm1Hyn targeted mutation die during embryonic development. Homozygous mutant embryos exhibit defects in the vasculature of the yolk sac and the embyro as well as severe defects in posterior and extraembryonic mesoderm. Implantation and initiation of gastrulation and neurulation is normal. There is normal development of notochord, somite and considerable development of brain, optic and otic anlagen and branchial arches. | ||
| 008120 | B6.129S4-Timp2tm1Pds/J | Cryopreserved - Ready for recovery |
| Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (mRNA) is detected by Northern blot analysis of lung tissue. Mutant mice are unable to proteolytically activate proMMP-2, proenzyme matrix metalloproteinase-2. Male homozygotes exhibit deficits in fear-potentiated startle respnses and both male and female mutants exhibit reduced prepulse inhibition. Homozygotes exhibit muscle weakness (due to reduced mass of extensor digitorum longus fast-twitch muscle), decreased motor function, abnormal gait and reduced hindlimb extension. The brains of homozygotes in the postnatal week are smaller in size than wildtype controls, with the size difference disappearing after postnatal day 7. Histological analysis reveals abnormal neuromuscular junctions characterized by a larger size with increased nerve branching, reduced cerebellar cortex thickness, and reduced Purkinje cell pr ..... For more information please see the full phenotype on the strain data sheet | ||
| 006943 | B6.129S7-B4galt1tm2Shur/J | Cryopreserved - Ready for recovery |
| These mice carry a mutant allele that has a point mutation in the first translation initiation codon in exon 1, which initiates translation of the long isoform of beta 1,4-galactosyltransferase. The second translation initiation codon in exon 1 is not affected. These mice express only the shorter isoform of beta 1,4-galactosyltransferase. No long isoform protein is detected in mammary tissue by Western blot analysis. Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Total Beta 1,4-galactosyltransferase activity is reduced to 72% of wildtype levels in mammary gland epithelial cells while activity on mammary epithelial cell surfaces is diminished by over 60%. Sperm and testis exhibit near wildtype levels of enzyme activity and glycoprotein galactosylation. The short isoform is expressed ectopically in sperm. Although able to undergo normal acrosomal exocytosis induced by calcium ionoph ..... For more information please see the full phenotype on the strain data sheet | ||
| 005514 | B6.129X1-Mmp8tm1Lotn/J | Cryopreserved - Ready for recovery |
| Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (protein) is detected by Western blot analysis of peripheral blood cells. Male mice homozygous for the mutation are more susceptible to experimentally induced invasive papillomas and fibrosarcomas. Latency of papilloma tumor formation is shorter. Female homozygotes exhibit increased skin cancer incidence when treated with tamoxifen or ovarectomy. Carcinogen administration causes persistent inflammatory response with neutrophil accumulation at the site of injection. Lipopolysaccharide (LPS) challenge causes a two-fold increase in polymorphonuclear cell accumulation in affected lung tissue of male homozygotes. This mutant mouse strain may be useful in studies of carcinogenesis, metastasis, and inflammation. | ||
| 007226 | B6;129P2-Has2tm1Jam/J | Cryopreserved - Ready for recovery |
| While heterozygous mice are viable and fertile, mice homozygous for this targeted allele die between embryonic day (E)9.5 and E10.5. Embryonic mRNA expression from the targeted gene shows only truncated transcripts of the expected length, with no full-length mRNA expression. Homozygous embryos exhibit severe cardiac and vascular abnormalities, lack hyaluronan (HA), and have yolk sac and somite deformities. Heart deformities can be rescued in explants from homozygous mice following exogenous HA or activated H-Ras treatment. These Has2 mutant mice may be useful in studying embryogenesis and development, specifically cardiac and vascular morphogenesis, as well as cell transformation. | ||
| 004234 | B6;129S-Fgfr3tm1Dor/J | Cryopreserved - Ready for recovery |
| Approximately 50% of mice that are homozygous for the Fgfr3tm1Dor targeted mutation die between birth and 21 days of age. Surviving pups may live as long as 8 months. RNAase protection analysis of adult homozygous brain tissue indicates that an abnormal transcript may be generated, but that no functional protein results. Severe skeletal defects (kyphosis, scoliosis, crooked tails, curvature and overgrowth of long bones) are evident. Inner ear defects (lack of pillar cell differentiation and tunnel of Corti formation) resulting in profound deafness are also observed. | ||
| 000063 | B6C3Fe a/a-sy/J | Cryopreserved - Ready for recovery |
| Most mice homozygous for the shaker-with-syndactylism mutation (sy) die within the first month, and none have lived to breed. Homozygous mutant mice may be syndactylous on all four feet; the forefeet may often be normal and possibly one or both hindfeet very occasionally so. The remainder of the skeleton shows many slight anomalies in addition to small size. The shafts of the long bones are considerably thinner than normal, and there are differences in shape of the sacral vertebrae and the scapula. Abnormal behavior appears during the first week. It consists of head-tossing and some circling, and the affected mice are always deaf. Abnormalities of the labyrinth can be seen at E13. | ||
| 000244 | C3Fe(B6)-Fbn2fp/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the recessive fused phalanges mutation (Fbn2fp) have syndactyly of the second, third, and/or the fourth digits of the hindlimbs and sometimes also the forelimbs, and this trait is usually not bilaterally symmetrical (Hummell and Chapman, 1971; Chaudhry et al., 2001). This syndactyly likely results from defective mesenchyme differentiation rather than failed interdigital apoptosis (Arteaga-Solis et al., 2001). Although western blot analysis fails to detect FBN2 in Fbn2fp homozygotes, rotary shadowing electron microscopy identifies abundant, morphologically normal microfibrils in adult skin and lung (Chaudhry et al., 2001). | ||
| 005710 | FVB.129S-Mmp13tm1Werb/J | Cryopreserved - Ready for recovery |
| Mice that are homozygous for this loxP-flanked ("floxed") allele are viable and fertile with normal endogenous gene function. Cre-mediated recombination results in replacement of exons 3-5 of targeted gene with a single loxP site. When bred to cre-expressing transgenic strains, these floxed mutant mice may be used to generate whole mouse or tissue-specific targeted mutants that may be useful in examining extracellular matrix remodeling and bone development. Of note: when these floxed mice are bred to mice containing a beta-actin promoter-driven cre transgene the resulting cre-positive, homozygous-null mice show robust accumulation of cartilage matrix caused by a transient expansion of the hypertrophic zone and increased trabecular bone mass that persists for months. | ||
| 006473 | STOCK Smyd1tm1Dsr/J | Cryopreserved - Ready for recovery |
| Mice heterozygous for this targeted mutation are viable and fertile. Mice homozygous for this targeted allele, however, die between embryonic day (E) 9.5 and 10.5 due to cardiomyocyte maturation defects, including an enlarged heart, single left-side ventricular chamber with tremendous extra cellular matrix expansion between the myocardial and endocardial layers, and defective Hand2 expression. No mRNA transcripts from the targeted mutant gene are detected in cardiac tissue from E9.5 homozygotes. These mutant mice may be useful in studying cardiomyocyte differentiation and cardiac morphogenesis. In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available. | ||
| 000596 | STOCK T(2;11)30H/+ x AEJ-a Gdf5bp-H/J or A/J-a Gdf5bp-J/J | Cryopreserved - Ready for recovery |
(17 stocks) Back to Top
How to Register Interest
Please indicate your interest in purchasing any of the strains listed below when they become available for distribution by checking the box next to the strain(s) of interest and then selecting the "Continue" button which leads to an Interest Form.View a Data sheet for New Strains Awaiting Transfer
Select the strain name to link to the strain data sheet.
Send questions to our Technical Support team using the Express Technical Support Form.
(5.3)