Search Criteria: Research Area is "Internal/Organ Research: Heart Abnormalities"
New Strains Awaiting Transfer from the Donor
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 002684 | B6.129P2-Nos3tm1Unc/J | Level 4 |
| Mice homozygous for the Nos3tm1Unc targeted mutation are viable and fertile. They have elevated blood pressure that is about 20 mmHg higher than that seen in normal wildtype siblings. They also show a decreased heart rate. Female homozygotes are smaller in body weight than normal wildtype siblings. Hyperglycemic-euglycemic clamp studies demonstrate that homozygotes exhibit insulin resistance at the level of the liver and peripheral tissues. | ||
| 008286 | 129S6.129P2(B6)-Nos3tm1Unc/J | Repository- Live |
| Homozygous mutant mice 129S6/SvEvTac background are viable, fertile, normal in size and do not exhibit any gross physical or behavioral abnormalities. On the C57BL/6 congenic background homozygous mutants mice exhibit elevated blood pressure that is about 20 mmHg higher than that seen in normal wildtype siblings. They also show a decreased heart rate. Female homozygotes are smaller in body weight than normal wildtype siblings. Hyperglycemic-euglycemic clamp studies demonstrate that homozygotes exhibit insulin resistance at the level of the liver and peripheral tissues. This mutant mouse strain may be useful in studies of wound healing, hypertension and other cardiovascular defects, insulin resistance, hyperlipidemia and lung development. 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. This is the case for the strain ..... | ||
| 013224 | B6.129P2-Abl1tm2.1Goff/J | Repository- Live |
| These Abl1flox/flox mutant mice posses loxP sites flanking exons 5-6 of the c-abl concogene 1, non-receptor tyrosine kinase gene, Abl1. Mice that are homozygous for this allele are viable, fertile, and normal in size. When these Abl1flox/flox mutant mice are bred to mice that express Cre recombinase, the resulting offspring will have exons 5-6 deleted in the cre-expressing tissue. This strain may be useful for studying the growth, development, and physiology of cardiac tissue. | ||
| 009125 | B6.129S1(Cg)-Lmnatm1Stw/BkknJ | Repository- Live |
| Mice heterozygotes for this lamin A/C mutation are viable and fertile. The targeted allele does not express both full-length transcripts or stable lamin A/C protein. Homozygotes (Lmna -/- mice) exhibit severely retarded postnatal growth beginning as early as 2 weeks of age and abnormal movement/gait by 3-4 weeks of age that progresses to distinct scoliosis/kyphosis and death around 8 weeks of age. Lmna -/- mice also have tissue-specific alterations of nuclear envelope integrity and mislocalization of the inner nuclear membrane protein emerin. In skeletal and cardiac muscle, this results in rapid myopathic onset closely resembling Emery-Dreifuss muscular dystrophy (EDMD). These mice are a model for the autosomal variant of EDMD and may be useful in studying the role of lamins, inner nuclear membrane proteins, nuclear envelope integrity, and chromatin domain anchoring sites in EDMD.
In an attempt to offer alleles on well-characterized or multiple genetic background ..... | ||
| 013787 | B6.129S1-Abcc6tm1Jfk/J | Repository- Live |
| Mice that are homozygous for this targeted mutation develop patchy, progressive mineralization of the dermis, kidneys, eyes, arteries, heart and subcutaneous tissues, mimicking the human disease symptoms of pseudoxanthoma elasticum. Mineralization of connective tissue capsule surrounding vibrissae is detectable at 5 weeks of age. Mineralization is associated with collagen fibrils and elastic fibers. Oxidative stress, as indicated by lipid peroxidation and protein oxidation, is higher in liver tissue and serum of mutant mice when compared to wildtype controls. Antioxidant diet reduces the oxidative stress levels in knock out mice, but does not prevent ectopic mineralization. The severity of mineralization can be altered by mineral intake as a diet with elevated magnesium levels prevents pathological connective tissue mineralization in mutants. Serum matrix gla protein (MGP) levels are reduced in homozygotes and the protein is under-carboxylated with diminished activity. Fetuin-A ..... For more information please see the full phenotype on the strain data sheet | ||
| 010620 | B6.129S1-Notch2tm1Grid/J | Repository- Live |
| Mice that are heterozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Due to alternative splicing, 2 in-frame gene products (mRNA) are detected by RT-PCR analysis of homozygous embryos. The mutant transcripts would produce proteins with one or two EGF repeats deleted. Levels of the mutant transcripts are similar to the wildtype transcript level. This targeted allele is a hypomorph. Homozygotes are neonatal lethal due to developmental defects in the kidney, heart and eye vasculature. Homozygous neonates exhibit hypoplastic kidneys, with vasculature lesions at the cortical surface, and lack mature glomeruli. Bilateral microphthalmia, with retrolenticular hyperplasia, is observed in homozygotes. At age embryonic day 11.5, some homozygous embryos exhibit delayed growth, pericardial effusion and widespread hemorrhaging. Homozygous embryos that survive past embryonic day 11.5 display myocardial hy ..... For more information please see the full phenotype on the strain data sheet | ||
| 006243 | B6.129S4-Timp1tm1Pds/J | Repository- Live |
| Homozygous females and hemizygous males (the gene is X-linked) are viable and fertile. No endogenous transcript is detected in lung tissue from affected mice by Northern blot analysis.. Homozygous mice have increased resistance to corneal and pulmonary infection with P. aeruginosa, and have altered immune, hematopoietic, and vascular permeability in bleomycin-induced lung injury trials. Homozygotes also show increased and continued progression of aneurysm formation compared with wild-type mice in induced thoracic aortic aneurysm (TAA) models. Mice with this X-linked targeted mutation may be useful in studies of cornea and pulmonary infection, pulmonary injury and aneurysm, as well as of P. aeruginosa resistance in individuals with unresolved, antibiotic-resistant pulmonary infections, such as those often observed in cystic fibrosis patients.
In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genet ..... | ||
| 005657 | B6.FVB(129)-Tg(Myh6-cre/Esr1*)1Jmk/J | Repository- Live |
| The alpha-MHC-MerCreMer transgene has the mouse Myh6 promoter (myosin, heavy polypeptide 6, cardiac muscle, alpha; alpha-MHC) directing expression of a tamoxifen-inducible Cre recombinase (MerCreMer) to juvenile and adult cardiac myocytes. Mice homozygous for the alpha-MHC-MerCreMer transgene are viable, fertile, normal in size, and do not display any gross physical or behavioral abnormalities. Cre recombinase expression in heart tissue is confirmed by western blot. Southern blot confirmed heart cell specificity compared to brain, kidney, lung, liver, and skeletal muscle. Insertion of this transgene and its protein show no changes in echocardiography, heart mass or pathology, or hypertrophy marker genes compared to nontransgenic littermates. Of note, the MerCreMer double fusion protein has substantially greater Cre recombinase activity with less promiscuity compared with the CreMer single fusion protein. When alpha-MHC-MerCreMer transgenic mice are bred with mice containing For more information please see the full phenotype on the strain data sheet | ||
| 008340 | BKS.Cg-Leprdb Nos3tm1Unc/RhrsJ | Repository- Live |
| These double mutant mice harbor both the Leprdb spontaneous mutation and the Nos3 (eNOS) targeted mutation and are congenic on C57BLKS/J genetic background. Mice homozygous for both mutations (called eNOS-/- C57BLKS/J db/db, eNOS-/- db/db, or db/db/eNOS-/- double mutant mice) are viable with development of type II diabetes and diabetic nephropathy. The eNOS-/- C57BLKS/J db/db mice develop type II diabetes with significant obesity and hypertension: while both mutations are associated with insulin resistance, the mutated leptin receptor in db/db mice chiefly accounts for the observed hyperglycemia and defective leptin signaling (leading to persistent hyperphagia and obesity), and the eNOS-deficiency accounts for the moderate systemic hypertension. In addition, eNOS-/- C57BLKS/J db/db mice exhibit rapid onset and progression of pathologic ..... For more information please see the full phenotype on the strain data sheet | ||
| 016211 | C57BL/6N-Agtr1atm1Uky/J | Repository- Live |
| These AT1aflox mutant mice possess a loxP site upstream of exon 3 followed by a neomycin resistance (neo) cassette flanked by frt sites and loxP sites downstream of exon 3 of the angiotensin II receptor, type 1a (Agtr1a) gene. Mice that are homozygous for this allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. AGTR1A is expressed in vascular cells such as endothelial cells, smooth muscle cells, and macrophages. Angiotensin II (Ang II) is a vasoconstrictor which, upon binding to AGTR1A, can induce aneurysms in the ascending aorta. When bred to mice that express tissue-specific Cre recombinase, resulting offspring will have exon 3 deleted in the cre-expressing tissues. When this floxed strain is crossed to a strain expressing Cre recombinase in smooth muscle, deletion of Agtr1a had no affect on ascending aortic aneurysms (AA) after Ang II infusion. In contrast, when cr ..... For more information please see the full phenotype on the strain data sheet | ||
| 017319 | FVB-Tg(Myh6-Mtpn)4Ssen/J | Repository- Live |
| 016571 | FVB-Tg(Myh6/tetO-Gata6)2Jmol/J | Repository- Live |
| These Gata6 transgenic mice contain the GATA binding protein 6 (Gata6) sequence regulated by a tetracycline operator (tetO), driven by myosin, heavy polypeptide 6, cardiac muscle, alpha (Myh6 or α-MHC) promoter/enhancer elements. Hemizygotes are viable, fertile, and normal in size. α-MHC limits overexpression of GATA6 to the heart. GATA6 is a zinc-finger-containing transcription factor expressed in mesoderm and endoderm derived tissues such as heart, liver, lung, gonad, and gut. Along with GATA4, GATA6 is necessary for the development of the embryonic heart and cardiac hypertrophy in the adult heart. When mated to a mutant strain expressing tetracycline-controlled transactivator protein (tTA), expression of GATA6 protein may be regulated with tetracycline or its analog doxycycline (dox) in the double mutant offspring. When bred to mice expressing tTA driven by the α-MHC promoter, double transgenic an ..... For more information please see the full phenotype on the strain data sheet | ||
| 014153 | FVB-Tg(Myh6/tetO-Itpr2)3.11Jmol/J | Repository- Live |
| These transgenic animals carry the mouse Itpr2 (inositol 1,4,5-triphosphate receptor 2) gene driven by a conditional Myh6 (myosin, heavy polypeptide 6, cardiac muscle, alpha)-tetO cardiac-specific promoter.
When crossed with a driver strain encoding the tetracycline transactivator (tTA), a 12-fold increase in protein expression is observed. Overexpression in the heart generates mild baseline cardiac hypertrophy at 3 months of age, but no greater signs of heart disease past 10 months of age. Increased hypertrophic response occurs following pathological/physiological stimuli. | ||
| 014154 | FVB-Tg(Myh6/tetO-Itpr2)4.9Jmol/J | Repository- Live |
| These transgenic animals carry the mouse Itpr2 (inositol 1,4,5-triphosphate receptor 2) driven by a conditional Myh6 (myosin, heavy polypeptide 6, cardiac muscle, alpha)-tetO cardiac-specific promoter.
When crossed with a driver strain encoding the tetracycline transactivator (tTA), a 5-fold increase in protein expression is observed. No phenotype is observed at baseline, but increased hypertrophic response occurs following certain types of stumuli. Administration of doxycline (Dox) causes near complete extinguishment of ITPR2 expression. | ||
| 006206 | FVB.129S6-Gt(ROSA)26Sortm2(HIF1A/luc)Kael/J | Repository- Live |
| Mice heterozygous for this "ODD-luc" knock-in are viable and fertile with no gross phenotypic or behavioral abnormalities. These mice have the C-terminal portion of the hypoxia-inducible factor 1 alpha (HIF1A) oxygen-dependent degradation domain (ODD) fused to the firefly luciferase (luc) gene. This region of the ODD also contains a proline residue (amino acid 564) that, when hydroxylated, will serve as a binding site for von Hippel-Lindau tumor suppressor protein (pVHL). Under normal oxygen concentrations, prolyl hydroxylation by egg-laying-defective nine (EGLN) proteins leads to pVHL-dependent polyubiquitylation and proteasomal degradation (thus, little or no luciferase fluorescence). Under hypoxia, proline hydroxylation is impaired and ubiquitination is attenuated, resulting in stabilization of the fusion protein and high levels of luciferase fluorescence in the hypoxic tissue(s). These "ODD-Luc" bioluminescent reporter mice may be useful in researching transcriptional ..... For more information please see the full phenotype on the strain data sheet | ||
| 012460 | FVB/N-Tg(Myh6-Gnaq)40Gwd/J | Repository- Live |
| Mice hemizygous for the Myh6-Gnaq allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. The donating investigator states that hemizygous females develop peripartum cardiomyopathy. Expression of Gαq is regulated by an α-myosin heavy chain (Myh6) promoter, which leads to overexpression of Gαq in the heart. This overexpression results in cardiac hypertrophy, defined as a conserved program of fetal gene expression, increased heart weight, and increased cardiomyocyte size, which severely compromises systolic cardiac function, and results in overt cardiac failure. Upon experimental pressure overloading, the mice progress rapidly to heart failure. These mice may be useful for studying the biochemical and physiologic phenotype resembling both the compensated and decompensated phases of human cardiac hypertrophy. | ||
| 010911 | STOCK Wt1tm1(EGFP/cre)Wtp/J | Repository- Live |
| Homozygous mice die between embryonic day (E)13.5 and birth with defects of heart, kidney, gonads, and multiple other organs. Heterozygous (Wt1GFPCre/+) mice are viable and fertile. The Wt1GFPCre "knock-in" allele both abolishes Wt1 gene function and expresses an enhanced green fluorescent protein-Cre recombinase fusion protein (EGFPCre) from the Wt1 promoter/enhancer elements. In heart from heterozygous mice, EGFPCre expression is directed to proepicardium and epicardium from E9.5 to E15.5, and is not found in the myocardium. When bred to mice containing loxP-flanked sequences, the resulting offspring will have Cre-mediated deletion of the floxed sequences in the Wt1-expressing cells (and their descendants). As Wt1 is expressed in the developing genitourinary system and in the mesothelia overlying most visceral organs, these mutant mice may be useful as fluorescent/Cre-lox tools for lineage-tracing/marking Wt1-expressin ..... For more information please see the full phenotype on the strain data sheet | ||
| 010912 | STOCK Wt1tm2(cre/ERT2)Wtp/J | Repository- Live |
| Homozygous mice die between embryonic day (E)13.5 and birth with defects of heart, kidney, gonads, and multiple other organs. Heterozygous (Wt1CreERT2/+) mice are viable and fertile. The Wt1CreERT2 "knock-in" allele both abolishes Wt1 gene function and has expression of the CreERT2 fusion protein (CreERT2) under control of the Wt1 promoter/enhancer elements. In heart from heterozygous mice, CreERT2 expression is directed to proepicardium and epicardium from E9.5 to E15.5, and is not found in the myocardium. CreERT2 fusion gene activity is inducible; observed following tamoxifen administration. As such, when Wt1CreERT2 mice are bred with mice containing loxP-flanked sequence, tamoxifen-inducible Cre-mediated recombination will result in deletion of the floxed sequences in the Wt1-expressing cells of the offspring. The donating investigator reports that Cre activity may be observed prior to tamoxifen exposure only in ..... For more information please see the full phenotype on the strain data sheet | ||
| 005650 | STOCK Tg(Myh6-cre/Esr1*)1Jmk/J | Repository- Live |
| The alpha-MHC-MerCreMer transgene has the mouse Myh6 promoter (myosin, heavy polypeptide 6, cardiac muscle, alpha; alpha-MHC) directing expression of a tamoxifen-inducible Cre recombinase (MerCreMer) to juvenile and adult cardiac myocytes. Mice homozygous for the alpha-MHC-MerCreMer transgene are viable, fertile, normal in size, and do not display any gross physical or behavioral abnormalities. Cre recombinase expression in heart tissue is confirmed by western blot. Southern blot confirmed heart cell specificity compared to brain, kidney, lung, liver, and skeletal muscle. Insertion of this transgene and its protein show no changes in echocardiography, heart mass or pathology, or hypertrophy marker genes compared to nontransgenic littermates. Of note, the MerCreMer double fusion protein has substantially greater Cre recombinase activity with less promiscuity compared with the CreMer single fusion protein. When alpha-MHC-MerCreMer transgenic mice are bred with mice containing For more information please see the full phenotype on the strain data sheet | ||
| 016572 | STOCK Tg(Myh6/tetO-Gata4)1Jmol/J | Repository- Live |
| These Gata4 transgenic mice contain GATA binding protein 4 (Gata4) sequence regulated by a tetracycline operator (tetO), driven by myosin, heavy polypeptide 6, cardiac muscle, alpha (Myh6 or α-MHC) promoter/enhancer elements. Hemizygotes are viable, fertile, and normal in size. α-MHC limits overexpression of Gata4 to the heart. GATA4 is a zinc-finger-containing transcription factor expressed in cardiomyocytes and has a role in regulating the expression of genes involved in cardiac differentiation. When mated to a mutant strain expressing tetracycline-controlled transactivator protein (tTA), expression of GATA4 protein may be controlled by the administration of tetracycline or its analog doxycycline in bi-allelic offspring. For instance, when bred to mice expressing tTA driven by the α-MHC promoter, double transgenic animals exhibit an increase in myocardial capillary densities, coronary flow reserve, and p ..... For more information please see the full phenotype on the strain data sheet | ||
| 009083 | 129S-Dvl3tm1Awb/J | Cryopreserved - Ready for recovery |
| 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 null mice have a perinatal lethal phenotype. Neonates have breathing difficulties and are often cyanotic. Homozygous embryos exhibit cardiac conotruncal abnormalities such as persistent truncus arteriosis (PTA) and double outlet right ventricle (DORV), and cochlear defects (disoriented stereociliary bundles). This mutant mouse strain may be useful in studies of cardiac development, neural tube formation and development of the inner ear. | ||
| 009085 | 129S/Sv-Rettm1Cos/J | Cryopreserved - Ready for recovery |
| The Ret- allele (also called ret-k-, ret-k minus, or c-ret-) disrupts the region of the ret proto-oncogene (Ret; also called ret-k or c-ret) locus harboring the invariant lysine codon required for Ret kinase activity. Homozygous mice die around 16-24 hours after birth, exhibiting abnormalities in kidney/urinary (renal agenesis/hypodysplasia) and peripheral nervous system development (including sympathetic, parasympathetic, and enteric ganglia), as well as abnormal enteric neural crest cell migration. Because homozygous mice lack enteric ganglia from the hindgut, these mice are also a model of Hirschsprung's Disease. | ||
| 013225 | B6(Cg)-Abl1tm1Goff/J | Cryopreserved - Ready for recovery |
| These Abl1m3 mutant mice possess a floxed neomycin resistance (neo) cassette, containing a stop codon, between exons 10 and 11 of the c-abl concogene 1, non-receptor tyrosine kinase gene, Abl1, resulting in low widespread detection of truncated protein. The allele also introduced five amino acid substitutions (Lysine to Glycine) in the first of three nuclear localization signals (NLS1) in exon 11. Mice that are heterozygous for this allele are viable, fertile, and normal in size. Homozygotes exhibit perinatal death with enlarged hearts. When these Abl1m3 mutant mice are bred to mice that express Cre recombinase, the resulting offspring will have the neo cassette deleted in the cre-expressing tissue, resulting in restoration of gene function while retaining the NLS1 mutations, and survival past birth. When bred to mice that express a cardiac-specific α-MHC-cre transgene (see Stock No. For more information please see the full phenotype on the strain data sheet | ||
| 005704 | B6.129-Fbn1tm2Rmz/J | Cryopreserved - Ready for recovery |
| Both heterozygous and homozygous "mgR" mutant mice are viable with no phenotypic abnormalities at birth. The protein expressed from this mutant allele is the same size as wild-type. Skin tissues show an intermediate reduction in transcript levels compared to wild-type and the mg-delta null allele. Thus the "mgR" mutation does not completely ablate gene function (resulting in rapid death). Instead, expression is hypomorphic and conducive to studying the clinical stages precursive to animal lethality. Homozygotes develop medial calcification, the inflammatory-fibroproliferative response, and inflammation-mediated elastolysis in the natural history of dissecting aneurysm and die between 2-6 month of age with Marfan syndrome (MFS)-like manifestations. | ||
| 004068 | B6.129-Iduatm1Clk/J | Cryopreserved - Ready for recovery |
| At birth, mice that are homozygous null for the Idua gene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No alpha-L-iduronidase enzyme activity or mRNA is detected. By three weeks of age, homozygous null mice develop a flattened facial profile and a thickening of digits. Defective bone formation is noticeable by fifteen weeks, characterized by a broadening and thickening of long bones. Evidence of lysosomal storage disorder is apparent in cells of the reticuloendothelial system at four weeks. By eight weeks progressive evidence of lysosomal storage is seen in Kupffer cells, splenic sinusoidal lining cells, chondrocytes, glial and Purkinje cells. This animal model is suitable for use in studies investigating the lysosomal storage disorder mucopolysaccharidosis type I (MPS I). | ||
| 003360 | B6.129-Juptm1Kem/J | Cryopreserved - Ready for recovery |
| Plakoglobin Jup null-mutant embryos die from embryonic day 10.5 onward due to severe heart defects. Some mutant embryos develop further, especially on a C57BL/6 background. These mice die around birth, presumably due to cardiac dysfunction, and with skin blistering and subcorneal acantholysis. The skin phenotype in plakoglobin-deficient mice is reminiscent of the human blistering disease, epidermolytic hyperkeratosis. | ||
| 003334 | B6.129-Juptm1Ruiz/J | Cryopreserved - Ready for recovery |
| Plakoglobin (gamma catenin) is a constituent of the cytoplasmic plaque of desmosomes as well as of other adhering cell junctions, and is involved in anchorage of cytoskeletal filaments to specific cadherins. Homozygous null mutant animals die between days 12 - 16 of embryogenesis due to defects in heart function. The tissue instability correlates with absence of desmosomes in the heart. | ||
| 003537 | B6.129-Kif3atm1Gsn/J | Cryopreserved - Ready for recovery |
| Ciliary formation appears to be crucial in developing left-right asymmetry in early mouse embryonic development. One of the necessary cilia components is the Kif3a gene product. Homozygous Kif3a knockout mice die at 10 days postcoitum, exhibit randomized establishment of left-right asymmetry and display numerous structural abnormalities. Cardiac looping is randomized, often retarded. Growth is severely retarded with caudal truncation. A neural tube closure defect is also apparent. Scanning electron microscopy indicates the absence of embryonic cilia. A small percentage of heterozygotes exhibit morphological abnormalities. | ||
| 003461 | B6.129P2(C)-Thratm1Ven/J | Cryopreserved - Ready for recovery |
| The Thra gene encodes the T3 receptor TRa1 (alpha 1) and a non-T3 bindng product TRa2 (alpha 2), generated by alternative splicing in the C-terminal region. This Thra mutant is deleted for the T3 receptor TRa1 (ALPHA ONE) but retains TRa2. These mutants have an average heart rate 20% lower than that of control animals, prolonged QTend and QRS durations, body temperatures 0.5 degrees C lower than that of control animals. A mild hypothyrodism is present but is restricted to males. | ||
| 002679 | B6.129P2-Acetm1Unc/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Acetm1Unc targeted mutation lack both the somatic and testicular isozyme forms of the Ace gene. They show reduced viability prior to weaning. Surviving homozygotes are hypotensive with blood pressures ~35 mmHg lower than normal wildtype silbings. Heterozygous male mice have blood pressures 15-20 mmHg below wildtype siblings. Fertility of homozygous males is greatly impaired. Ten to twelve month old female homoygotes exhibit abnormal renal vessels and tubules, increased renin synthesis accompanied by an abnormal expression pattern. | ||
| 004159 | B6.129P2-Adra1btm1Cta/J | Cryopreserved - Ready for recovery |
| Mice that are homozygous null for the targeted allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No Adra1b transcripts are detected. The amount of alpha 1 adrenergic recptor binding activity as measured by radioligand assays is dramatically decreased in homozygous liver, heart and cerebral cortex. The blood pressure response induced by increasing doses of phenylephrine is reduced by 45% in comparison to wild type mice. Similarly, phenylephrine-induced contraction of aortic tissue is diminished 25%. This mutant mouse strain represents a model that may be useful in studies related to blood pressure regulation. | ||
| 006620 | B6.129P2-Scp2tm1Usee/J | Cryopreserved - Ready for recovery |
| Mice homozygous for this targeted mutation are viable and fertile and do not display any gross physical or behavioral abnormalities. No protein product from the targeted gene is detected in liver tissue. Northern blot experiments show a low-intensity signal from a non-functional truncated transcript, however. Histologically, greater numbers of peroxisomes are observed in the livers of these mice. Liver function appears normal based on liver enzyme levels, but cholesterol and triglyceride storage pools are depleted. Hepatic gene expression is altered. Higher expression levels of liver fatty acid binding protein and multiple peroxisomal beta-oxidation enzymes are observed. Whereas plasma insulin and cholesterol concentrations are normal, triglycerides are slightly higher and free fatty acid and glucose concentrations are moderately lower in homozygous mice. Food intake is significantly higher in homozygotes as compared to control animals. A prononounced accumulation of phytanic acid is o ..... For more information please see the full phenotype on the strain data sheet | ||
| 002463 | B6.129S-Itga4tm1Hyn/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Itga4tm1Hyn targeted mutation die during embryonic development. Homozygous mutant embryos fail to fuse the allantois with the chorion during placentation. There is a defect in the epicardium and coronary vessels results in in utero cardiac hemorrhage; also known as CD49D, VLA-4. | ||
| 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. | ||
| 009387 | B6.129S1-Osr1tm1Jian/J | Cryopreserved - Ready for recovery |
| The Osr1tm1Jian (Odd1-LacZ) mutation abolishes endogenous gene function and expresses a β-galactosidase fusion protein (fused in-frame with the N-terminal 16 amino acid residues of the endogenous protein). Under control of the upstream promoter/enhancer elements, lacZ expression is directed to the same tissues as the wild-type gene (for example, β-galactosidase expression during embryogenesis is detected at E7.5 in the intermediate mesoderm, is expanded to the gut endoderm, lung bud mesenchyme and myocardial cells by E9.5, and is activated in developing branchial arches and limb buds by E10.5). Almost all (`95%) of homozygous mice die in utero between E11.5-E12.5 from circulation distress; exhibiting malformed atrial septum, dilated atria with hypoplastic venous valves, and blood backflow from the heart into systemic veins. Homozygotes also exhibit complete agenesis of adrenal glands, metanephric kidneys, gonads, and defects in pericard ..... For more information please see the full phenotype on the strain data sheet | ||
| 002719 | B6.129S4-Wt1tm1Jae/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Wt1tm1Jae targeted mutation die between embryonic days 13 and 15. They fail to develop a kidney or gonads. The hearts of homozygous mutant mice also fail to develop properly. Hearts are smaller than wildtype controls and possess a rounded apex. The right ventricular wall is thin and the left ventricle is reduced in size. There appears to be normal development of the aortic, pulmonary, mitral and tricuspid valves. Development of the diaphragm is also incomplete resulting in incomplete separation of the thoracic and abdominal cavities. Homozygous mutant lungs are also markedly smaller than wild type lungs. Heterozygous mice appear normal and show no tumor development (mice followed until 10 months of age). | ||
| 002646 | B6.129S6-Nf1tm1Fcr/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Nf1tm1Fcr targeted mutation die during embryonic development due to severe heart malformation (~E13). They also show hyperplasia of neural crest-derived sympathetic ganglia. Heterozygotes do not exhibit any overt disease symptoms. However, as noted in a another targeted mutation deleting the same exon of the Nf1 gene (Jacks, et al., Nat Genetics 7:353-361, 1994), they do show a predisposition to many types of tumors and were recently shown to have deficits in learning and memory (Silva, et al., Nat Genetics 15:281-284, 1997). | ||
| 000535 | B6.Cg-Atp7aMo-blo/J | Cryopreserved - Ready for recovery |
| Female mice heterozygous for the blotchy alleles (Atp7aMo-blo) are viable and fertile. They have irregular patches of light-colored fur. Hemizygous males and homozygous females have reduced viability and many are infertile. Hemizygotes and homozygotes are light all over with no blotching, are usually small, and occasionally have deformed hindlegs. Most hemizygotes and homozygotes have defective elastin in the aorta and usually die with aortic aneurysm. Hemizygous males have enlarged air spaces in the lung (emphysema), probably because of defective elastin and collagen. Skin collagen and aortic elastin have defective crosslinking at the step at which lysine residues are converted to aldehydes. Hemizygous males have a deficiency of noradrenalin in the brain, probably because a deficiency of copper shown to exist in the brain causes defective activity of the enzyme dopamine-beta-hydroxylase. Copper absorption from the gut and hepatic copper concentration are reduced to 6 ..... For more information please see the full phenotype on the strain data sheet | ||
| 008780 | B6.Cg-Hcn4tm1Rsei/J | Cryopreserved - Ready for recovery |
| These mice carry the HCN4R699Q mutation which results in an amino acid substitution in the cyclic nucleotide (cAMP)-binding domain of the protein. 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 mice have an embryonic lethal phenotype, developing normally until embryonic day 11 but then failing to develop past embryonic day 12. Western blot analysis of embryonic hearts indicates that the mutation does not alter the level of gene product (protein) expression. Isolated embryonic hearts from homozygous and heterozygous animals younger than embryonic day 11.5 have decreased heart rates when compared to wild-type. Homozygous embryonic hearts do not respond with increased heart rate to adrenergic stimulation. Cardiomyocytes isolated from homozygous embryos beat more slowly, and have slower current activation and faster current deactivation when compared to contro ..... For more information please see the full phenotype on the strain data sheet | ||
| 012389 | B6.Cg-Tg(Myh6-Ppara)404-3Dpk/J | Cryopreserved - Ready for recovery |
| The mouse Myh6 (myosin, heavy polypeptide 6, cardiac muscle, alpha) cardiac muscle-specific promoter drives expression of FLAG-tagged Ppara (peroxisome proliferator activated receptor alpha) in this transgenic strain. Expression of Ppara is increased approximately 135-fold as compared to wildtype in this line (404-3). Hearts of hemizygotes depend on fatty acid for the generation of energy to a significantly greater extent than do hearts of non-transgenic mice and have a substrate utilization profile similar to that of the diabetic heart. Cardiac dysfunction is exhibited by 2 months of age with no imposed stress. This strain may be useful in studies of metabolic alterations associated with diabetic cardiomyopathy. | ||
| 012382 | B6.Cg-Tg(Myh6-Ppara)404-4Dpk/J | Cryopreserved - Ready for recovery |
| The mouse Myh6 (myosin, heavy polypeptide 6, cardiac muscle, alpha) cardiac muscle-specific promoter drives expression of FLAG-tagged Ppara (peroxisome proliferator activated receptor alpha) in this transgenic strain. Expression of Ppara is increased approximately 15-fold as compared to wildtype in this line (404-4). Hearts of hemizygotes depend on fatty acid for the generation of energy to a significantly greater extent than do hearts of non-transgenic mice and have a substrate utilization profile similar to that of the diabetic heart. Cardiac dysfunction is exhibited by 4-5 months of age with no imposed stress, or after being placed on a high fat diet. This strain may be useful in studies of metabolic alterations associated with diabetic cardiomyopathy and other forms of lipotoxic cardiomyopathy. | ||
| 002201 | B6;129-Gja1tm1Kdr/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Gja1tm1Kdr targeted mutation die at birth. The cause of death is a failure in pulmonary gas exchange caused by a swelling and blockage of the right ventricular outflow tract from the heart. The cardiac abnormality involves a delay in the normal looping of the ascending limb of the heart tube, which includes the right ventricle and the outflow tract. This predisposes homozygous mutant mice to malformations of the subpulmonary outflow tract and tricuspid valve. The mutation also predisposes mice to lens cataracts and causes a severe reduction of germ cell numbers in homozygous mutant mice of both sexes. Both neonatal and adult mice heterozygous for the Gja1tm1Kdr targeted mutation have slower ventricular epicardial conduction of paced beats in the heart. | ||
| 009599 | B6;129P2-Adam19Gt(Betageo)1Bbl/J | Cryopreserved - Ready for recovery |
| Heterozygous mice are viable and fertile, while ~80% of homozygous mice die in the first few days after birth with severe heart valve defects. This β-geo secretory trap mutation abolishes endogenous gene function and expresses an ADAM19/LacZ/neo fusion protein. The mutant fusion protein has improper protein folding that keeps the ADAM19 regions of the protein retained in the endoplasmic reticulum by chaperones where they are subsequently degraded. As such, lacZ expression is directed to the same tissues as the wildtype gene. No wildtype ADAM19 protein product is observed from the targeted allele. These Adam19-mutant mice may be useful as a lacZ reporter for Adam19 expression or as a knockout model for studying developmental biology (cardiac morphogenesis). | ||
| 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. | ||
| 006470 | B6;129S-Hopxtm1Eno/J | Cryopreserved - Ready for recovery |
| Homozygous mice are viable and fertile on this mixed genetic background. Absence of the targeted protein is confirmed in heart and brain tissues from homozygotes. The lacZ expression pattern is similar to that of the endogenous gene. Homozygous heart tissues show altered serum response factor (SRF)-associated gene expression. Mice homozygous for this null allele segregate into two phenotypic classes characterized by an excess or deficiency of cardiac myocytes. These mutant mice may be useful in studying cardiac growth and development. | ||
| 008751 | B6;129S4-Nhlh1tm1Irk/J | Cryopreserved - Ready for recovery |
| Although mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities, approximately 25% will die prematurely before 1 year of age. No gene product (mRNA) is detected by Northern blot analysis of embryonic mouse heads. Premature death of homozygotes may be due to cardiac arrhythmia. Homozygotes have cardiac repolarization abnormalities, stress induced irregular heartbeat and reduced total heart rate power (index of heart rate variability). Impairment of the parasympathetic nervous system may cause the observed absence of diving reflex, reduced baroreceptor sensitivity and heart rate variability. This mutant mouse strain may be useful in studies of cardiac disease and cardiovascular physiology. | ||
| 002332 | B6;129S4-Wt1tm1Jae/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Wt1tm1Jae targeted mutation die between embryonic days 13 and 15. They fail to develop a kidney or gonads. The hearts of homozygous mutant mice also fail to develop properly. Hearts are smaller than wildtype controls and possess a rounded apex. The right ventricular wall is thin and the left ventricle is reduced in size. There appears to be normal development of the aortic, pulmonary, mitral and tricuspid valves. Development of the diaphragm is also incomplete resulting in incomplete separation of the thoracic and abdominal cavities. Homozygous mutant lungs are also markedly smaller than wild type lungs. Heterozygous mice appear normal and show no tumor development (mice followed until 10 months of age). | ||
| 003266 | B6;129S7-Epas1tm1Rus/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Epas1tm1Rus targeted mutation develop normally until embryonic day 11.5. Beginning at embryonic day 12.5 the ratio of homozygous embryos begins to decline and by day 16.5 there are no viable mutant embryos. Overall morphological development, including the circulatory system, is normal. Of particular interest however, is a pronounced bradycardia in homozygous mutant mice. Catecholamine levels in homozygous mutant mice are also significantly lower than wildtype controls. Administration of the catecholamine precursor DOPS to pregnant females rescues approximately 40% of mutant embryos. Embryos that survive to birth appear runted, fail to nurse, and die within 24 hours of birth. These results suggest a pivotal role of EPAS1 in catecholamine homeostasis. Heterozygous mice from this strain contain a modified lacZ gene in the targeting construct. This characteristic makes this strain useful as a marker for endothelial cells. | ||
| 002640 | B6;SJL-Tg(Myh6-ADRBK1)27Wjk/J | Cryopreserved - Ready for recovery |
| Mice carrying this transgene overexpress a truncated form of the bovine beta-adrenergic receptor kinase-1 containing only the last 194 amino acids of the ADRBK1 protein. The truncated protein inhibits endogenous beta-adrenergic receptor kinase-1 by blocking beta-adrenergic receptor desensitization. This myocardial inhibition increases cardiac function of homozygotes under basal conditions and leads to supersensitization to beta-agonists. This strain may serve as a model for increased left ventricular performance, a novel method of positive intropy, and enhanced beta-adrenergic signalling. | ||
| 012383 | B6CBA-Tg(Myh6-Ppard)HEDpk/J | Cryopreserved - Ready for recovery |
| The mouse Myh6 (myosin, heavy polypeptide 6, cardiac muscle, alpha) promoter drives expression of Ppard (peroxisome proliferator activator receptor delta) in these transgenic mice. Hemizygotes have increased myocardial glucose utilization, do not accumulate myocardial lipid, and have normal cardiac function. Expression levels in this "high expression" (HE) line are increased approximately 100-fold over endogenous levels. This strain may be useful in studies of cardiac function and metabolic modulation during diabetes and ischemia. | ||
| 002044 | B6Ei.Cg-Atp7aMo-blo/J | Cryopreserved - Ready for recovery |
| Female mice heterozygous for the blotchy alleles (Atp7aMo-blo) are viable and fertile. They have irregular patches of light-colored fur. Hemizygous males and homozygous females have reduced viability and many are infertile. Hemizygotes and homozygotes are light all over with no blotching, are usually small, and occasionally have deformed hindlegs.. Most hemizygotes and homozygotes have defective elastin in the aorta and usually die with aortic aneurysm. Hemizygous males have enlarged air spaces in the lung (emphysema), probably because of defective elastin and collagen. Skin collagen and aortic elastin have defective crosslinking at the step at which lysine residues are converted to aldehydes. Hemizygous males have a deficiency of noradrenalin in the brain, probably because a deficiency of copper shown to exist in the brain causes defective activity of the enzyme dopamine-beta-hydroxylase. Copper absorption from the gut and hepatic copper concentration are reduced to ..... For more information please see the full phenotype on the strain data sheet | ||
| 002639 | B6SJL-Tg(Myh6-ADRBK1)12Wjk/J | Cryopreserved - Ready for recovery |
| Mice carrying this transgene show a 3 to 5 fold overexpression of bovine beta-adrenergic receptor kinase-1. Myocardial beta-adrenergic receptors are hyperphosphorylated and desensitized in hemizygotes resulting in attenuated cardiac function in response to beta-agonists. | ||
| 002638 | B6SJL-Tg(WTbeta2)4Wjk/J | Cryopreserved - Ready for recovery |
| These transgenic mice exhibit increased basal myocardial adenylyl cyclase activity, enhanced atrial contractility, and increased left ventricular function in vivo; these parameters at baseline in the transgenic animals were equal to those observed in control animals maximally stimulated with isoproterenol. Mice carrying this transgene display a marked overexpression of the human beta-2 adrenergic receptor (~200 fold increase). | ||
| 013632 | C57BL/6-b2b019Clo/J | Cryopreserved - Ready for recovery |
| This undefined b2b019Clo mutation was identified in an ENU screen for recessive cardiovascular development phenotypes in Dr. Cecilia Lo's laboratory, NHLBI Cardiovascular Development Consortium (CvDC). It was recovered from G1 sperm and associated with the phenotype described here. Because G1 sperm were cryopreserved, additional incidental mutations are also segregating in this strain.
Homozygotes demonstrate cardiovascular defects that involve a double outlet right ventricle (DORV) and ventricular septal defect (VSD). Cleft palate and micrognathia (abnormally small lower jaw) are also seen. | ||
| 013633 | C57BL/6J-Bicc1b2b222Clo/J | Cryopreserved - Ready for recovery |
| This c.10048T->C Bicc1 (bicaudal C homolog 1 (Drosophila)) recessive mutation was identified in an ENU screen for recessive cardiovascular development phenotypes in Dr. Cecilia Lo's laboratory, NHLBI Cardiovascular Development Consortium (CvDC). It was recovered from G1 sperm and associated with the phenotype described described here. Because G1 sperm were cryopreserved, additional ENU mutations are also segregating in this strain.
Homozygotes demonstrate laterality defects, including situs inversus totalis, and heterotaxy. Congenital heart disease (CHD) is marked by double outlet right ventricle (DORV)/transposition of the great arteries (TGA), atrioventricular septal defects (AVSD), and an interrupted aortic arch (IAA). Polycystic kidney disease, as well as pancreatic, choleductal, and gonadal cysts are also found. | ||
| 013617 | C57BL/6J-b2b288Clo/J | Cryopreserved - Ready for recovery |
| This undefined b2b288Clo mutation was identified in an ENU screen for recessive cardiovascular development phenotypes in Dr. Cecilia Lo's laboratory, NHLBI Cardiovascular Development Consortium (CvDC). It was recovered from G1 sperm and associated with the phenotype described here. Because G1 sperm were cryopreserved, additional incidental mutations are also segregating in this strain.
Homozygotes demonstrate cardiovascular defects that involve a double outlet right ventricle (DORV), transposition of great arteries, dextrocardia, and complete atroventricular septal defect (AVSD). Heterotaxia is also seen. | ||
| 013618 | C57BL/6J-b2b386Clo/J | Cryopreserved - Ready for recovery |
| This undefined b2b386Clo mutation was identified in an ENU screen for recessive cardiovascular development phenotypes in Dr. Cecilia Lo's laboratory, NHLBI Cardiovascular Development Consortium (CvDC). It was recovered from G1 sperm and associated with the phenotype described here. Because G1 sperm were cryopreserved, additional incidental mutations are also segregating in this strain.
Homozygotes demonstrate cardiovascular defects that involve a double outlet right ventricle (DORV), common atrioventricular (AV) valve, duplicated inferior vena cava (IVC), interrupted/hypoplastic aortic arch, ventricular septal defect (VSD), and interrupted aortic arch (IAA). Left lung isomerism, left liver isomerism, and cleft palate are also seen. | ||
| 005863 | C57BL/6J-Tg(ACTB-DDAH1)1Jpck/J | Cryopreserved - Ready for recovery |
| Hemizygous mice are viable and fertile with no anatomic abnormalities. Transgene expression is observed in aorta, heart, and brain. Transgenic dimethylarginine dimethylaminohydrolase (DDAH) activity is reflected in a reduction of plasma asymmetric dimethylarginine (ADMA). Nitric oxide synthase (NOS) activity is significantly increased in transgenic skeletal and cardiac muscle. Transgenic mice have lower blood pressure and higher heart rate. Transgenic mice may be useful in studies of atherosclerosis, hypertension, hyperlipidemia, hypercholesterolemia, hyperhomocystinemia, diabetes mellitus, stroke, renal failure, preeclampsia, and other conditions associated with vascular pathophysiology and/or cardiovascular disease. | ||
| 007073 | CByJ.129P2(B6)-Nos3tm1Unc/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Nos3tm1Unc targeted mutation are viable and fertile. They have elevated blood pressure that is about 20 mmHg higher than that seen in normal wildtype siblings. They also show a decreased heart rate. Female homozygotes are smaller in body weight than normal wildtype siblings. Hyperglycemic-euglycemic clamp studies demonstrate that homozygotes exhibit insulin resistance at the level of the liver and peripheral tissues. This mutant mouse strain may be useful in studies of wound healing, hypertension and other cardiovascular defects, insulin resistance, hyperlipidemia and lung development.
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. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain de ..... | ||
| 006817 | D2.129S4(Cg)-Wt1tm1Jae/EiJ | Cryopreserved - Ready for recovery |
| 005057 | FVB.129-Kcnj2tm1Swz/J | Cryopreserved - Ready for recovery |
| Mice that are homozygous for the targeted mutation have a complete cleft of the secondary palate and die within 12 hours of birth. Heterozygotes are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (mRNA or protein) is detected by Northern blot analysis of cardiac tissue or Southern blot analysis. Inwardly rectifying potassium ion currents are absent in cerebral artery myocytes and cardiac ventribular myocytes isolated from homozygote neonates. Elevated external potassium ion concentrations do not dilate isolated neonatal cerebral arteries. Homozygotes exhibit altered electrocardiogram profiles indicative of reduced heart rate and bradycardia. This mutant mouse strain may be useful in studies of potassium ion dependent vasodilation, cardiac arrythmia such as Anderson syndrome, cleft palate and developmental bone malformation. | ||
| 002535 | FVB/N-Tg(ANX6)2Agh/J | Cryopreserved - Ready for recovery |
| Mice carrying the human annexin VI transgene display a phenotype similar to congestive heart failure. They show alterations in the contractility and calcium dynamics in cardiomyocytes, and these cells also have reduced basal calcium levels. Mice homozygous for the transgene die by 4 weeks of age while hemizygous mice live to about 1 year of age. | ||
| 008716 | FVB/N-Tg(Myh6-AIP/PLN*)46Jded/J | Cryopreserved - Ready for recovery |
| SR-AIP transgenic mice carry the α-MHC-AIP4-SR transgene. Hemizygous SR-AIP mice are viable and fertile, with expression of AIP4 (a tetramer of the CaMKII autocamtide inhibitory peptide AIP) directed to the sarcoplasmic reticulum (SR) of the heart by the murine alpha-myosin heavy chain (α-MHC or Myh6) promoter and a truncated phospholamban (PLB) transmembrane domain (harboring two loss-of-function mutations to prevent direct SERCA inhibition by the fusion protein). FLAG epitope expression may be used to identify presence of the fusion protein. The SR-targeted AIP concatemer peptide binds CaMKII, preventing phosphorylation of PLB and subsequent activation of SERCA, thereby increasing diastolic intracellular calcium. Whole
heart function and diastolic relaxation are slightly impaired, and pregnancy leads to earlier onset of cardiac hypertrophy. These SR-AIP transgenic mice may be useful in studying sarcoplasmic reticulum-targeted CaMKII inhibitio ..... For more information please see the full phenotype on the strain data sheet | ||
| 012461 | FVB/N-Tg(Myh6-Cast)1Gwd/J | Cryopreserved - Ready for recovery |
| Mice hemizygous for the Myh6-Cast allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Expression of Cast is regulated by an α-myosin heavy chain (Myh6) promoter, which results in the overexpression of calpastatain, a highly specific global inhibitor of calpain 1 and calpain 2, in the heart. Overexpression of calpastatin causes diminished ubiquitination of myocardial proteins resulting in a progressive dilated cardiomyopathy characterized by accumulation of intracellular protein aggregates, formation of autophagosomes, and degeneration of sarcomeres. These Myh6-Cast mice may be useful for assessing the consequences of cardiomyocyte-specific calpain inhibition in normal, ischemic, and failing hearts, and the contribution of endogenous calpains to myocardial protein turnover. | ||
| 000220 | LPT/LeJ | Cryopreserved - Ready for recovery |
| 004083 | NOD.129(B6)-Prkdcscid Iduatm1Clk/J | Cryopreserved - Ready for recovery |
| At birth, mice that are homozygous null for the Idua gene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No alpha-L-iduronidase enzyme activity or mRNA is detected. By three weeks of age, homozygous null mice develop a flattened facial profile and a thickening of digits. Defective bone formation is noticeable by fifteen weeks, characterized by a broadening and thickening of long bones. Evidence of lysosomal storage disorder is apparent in cells of the reticuloendothelial system at four weeks. By eight weeks progressive evidence of lysosomal storage is seen in Kupffer cells, splenic sinusoidal lining cells, chondrocytes, glial and Purkinje cells. This animal model is suitable for use in studies investigating the lysosomal storage disorder mucopolysaccharidosis type I (MPS I). | ||
| 003179 | STOCK Cdh2tm1Hyn/J | Cryopreserved - Ready for recovery |
| Mice that are heterozygous for the mutant allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. However, homozygous mice have an embryonic lethal phenotype, and die by embryonic day 10. The somites of the mutant embryos are small, irregularly shaped, and less cohesive compared with those of their wild-type littermates, and the epithelial organization of the somites is partially disrupted. Undulation of the neural tube is also observed in the mutant embryos. The mesodermal and endodermal cell layers of the yolk sac are separated in the mutants. The most dramatic cell adhesion defect is observed in the primitive heart; although myocardial tissue forms initially, the myocytes subsequently dissociate and the heart tube fails to develop normally. | ||
| 007863 | STOCK Dnahc5hlb612/JClo | Cryopreserved - Ready for recovery |
| The ENU generated hlb612 allele contains an in-frame deletion in a dynein gene (Dnahc5) commonly associated with human primary ciliary dyskinesia (PCD). Thirty-six percent of homozygotes exhibit situs inversus totalis and hydrocephaly and die between 2-4 weeks of age. Forty percent of homozygotes die before or shortly after birth and exhibit heterotaxy with structural heart defects and cardiovascular anomalies including discordant atrioventricular and ventricular outflow situs, atrial/pulmonary isomerisms, artery alignment defects, interrupted inferior vena cava and dextrocardia. Electronmicroscopy reveals that the outer dynein arms of the respiratory cilia are greatly reduced in number. Respiratory cilia exhibit a wide variation in orientation. Cilia in the airway epithelia are immotile or slow and dsykinetic. Heterozygous mice do not have situs defects, however, respiratory cilia exhibit some reduction in the number of outer dynein arms.
This strain may be use ..... For more information please see the full phenotype on the strain data sheet | ||
| 002906 | STOCK Mecomtm1Mmor/J | Cryopreserved - Ready for recovery |
| Heterozygous (Evi1+/-) mutant embryos die at E10.5 due to widespread hypocellularity, hemorrhaging, and disruption in the development of paraxial mesenchyme. In addition, defects in the heart, somites, and cranial ganglia were detected and the peripheral nervous system fails to develop. | ||
| 006578 | STOCK Myoz2tm1Eno/J | Cryopreserved - Ready for recovery |
| Mice homozygous for this calsarcin-1 mutant allele are viable and fertile. Immunoblot of homozygous cardiac tissue shows no endogenous protein expression. Strong lacZ expression throughout all cardiac chambers mirrors the expression pattern of the endogenous gene, and marked skeletal muscles known to contain a high proportion of type I (slow) fibers. Homozygotes have skeletal muscle abnormalities in type I (slow) fibers and calcineurin activity. Echocardiography of homozygous mice reveals abnormal heart performance. Absence of gene function activates a cardiac hypertrophic fetal gene program (despite the absence of hypertrophy) and enhanced the cardiac growth response to pressure overload. These mutant mice may be useful in studying growth and gene expression of cardiac and skeletal muscle, as well as the pathogenesis of human cardiomyopathies. In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic bac ..... | ||
| 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. | ||
| 008779 | STOCK Thtm1Srt/J | Cryopreserved - Ready for recovery |
| Mice that are heterozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. A small amount of gene product (protein) is detected by Western blot analysis of heads of homozygotes embryonic day 14.5 in age. Homozygous mice have an embryonic lethal phenotype, begin to die at embryonic day 11.5 and fail to develop past embryonic day 14.5. Homozygous embryos exhibit abnormal heart development with dilated atria, thin atrial walls, ventricular hypoplasia and blood congestion, dying with symptoms of congestive heart failure. Catecholamine containing cells are not detected by glyoxylic acid-induced histofluorescence analysis of embryos. Administration of L-Dopa or +/- isoproterenol to pregnant heterozygous females rescues approximately 90% of the homozygous pups, which then survive up to 3 weeks after birth. Homozygous pups can also be rescued by exposuring the pregnant dam to high oxygen (33-60%). Norepinephrine, ..... For more information please see the full phenotype on the strain data sheet | ||
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