You can find more information about these JAX® Mice strains and thousands of others by searching the JAX® Mice Database. To learn how to submit your unique research strain(s) to our international repository for mouse models, see our Strain Submission Form.

B6.129P2-Sncgtm1Vlb/J (008843) These mice harbor a null mutation of the γ-synuclein gene (Sncg) and may be useful in studying synuclein function in neurodegenerative diseases, such as Parkinson's disease.

STOCK Tg(THY1-SNCA*A53T)F53Sud/J (008474) These mice are transgenic for the A53T mutation of the human SNCA (synuclein, alpha) gene under the control of a human THY1 (thymus cell antigen 1, theta) promoter. Hemizygotes are viable and fertile and develop a Parkinson-like phenotype upon aging. Hind limb paralysis due to loss of motor neurons and a resting tremor are initially seen at about eight months of age. No Lewy body-like pathology is noted. Cell death in the spinal cord (extensive) and brain are observed.

Alzheiemer's Disease Mouse Models
A popular JAX® Mice strain (004462) is now more widely available. Researchers have used this strain to research Alzheimer's disease, amyloid plaque formation, and aging. Learn more about this strain and check out a table comparing Alzheimer's disease mouse models.

JAX® Aging Service
Study-ready animals for your age-related disease research. In some mouse models, a disease condition develops only with age. JAX® Aging Service will maintain your research strain, or any JAX® mice strain, and deliver mice to you at ages appropriate for your research projects. The cost for this service is based on strain, number of mice needed, and duration of aging.

B6.Cg-Tg(Pomc-MAPT/GFP*)1Rck/J (008322) This strain expresses a tau (MAPT)-sapphire green fluorescent protein (GFP) under control of the mouse pro-opiomelanocortin-alpha (Pomc) promoter. Immunohistochemistry for POMC in colchicine-treated mice demonstrated greater than 99% colocalization of POMC with the GFP-expressing neurons in the hypothalamic arcuate nucleus. This strain has been useful in studying the role of Pomc and leptin in food intake and may be used to further understand the function of the associated neurons. Hemizygotes are viable, fertile, and look and behave normally.

B6.Cg-Tg(Prnp-ITM2B/APP695*42)A12Emcg/J (007182) This BRI-Abeta42 transgenic harbors a mouse prion promoter-driven BRI-Abeta42 fusion construct (containing a human type 2 transmembrane protein (BRI or ITM2B) fused in-frame with a "wild-type APP695" cDNA sequence encoding amyloid-beta42 (Abeta42) at the furin-like cleavage site (the C-terminal 23 amino acid ABri peptide of BRI was replaced with the Abeta42 sequence)). As Abeta1-42 is fused to the C terminus of the BRI protein at the furin-like cleavage site, cleavage releases Abeta into the lumen or extracellular space, resulting in efficient secretion of Abeta1-42. Therefore, this strain expresses the Abeta1-42 isoform in the absence of human amyloid beta protein precursor (APP) overexpression and may be used to study Alzheimer's disease, neurodegeneration, and amyloid plaque formation.

B6.SJL-Slc6a3tm1.1(cre)Bkmn/J (006660) This dopamine transporter IRES-cre (DATIREScre) mutant strain exhibits co-localization of Cre recombinase and endogenous solute carrier family 6 (Slc6a3) (neurotransmitter transporter, dopamine), member 3) gene expression and may be used to study gene function in dopaminergic neurons in neurological diseases, such as drug addiction and Parkinson's disease.

Atherosclerosis: The Jackson Laboratory offers diet-induced and heritable mice for atherosclerosis research. One of the most relevant models is B6.129P2-Apoetm1Unc/J (002052). This ApoE-deficient strain is hypercholesterolemic and spontaneously develops arterial lesions.

Metabolic Syndrome: A promising model for metabolic syndrome is B6.129S7-Ldlrtm1Her/J (002207). This strain exhibits elevated serum cholesterol. Its cholesterol levels rise even higher when it is fed a high-fat diet. When fed a diabetogenic diet, these mice become obese and develop hypertriglyceridemia, hyperinsulinemia, moderate hyperglycemia, high vascular calcification, atherosclerotic plaques, and high free fatty acid levels.

Blood Pressure, Heart Disorders: We offer numerous JAX® Mice exhibiting elevated blood pressure, as well as mice with a variety of heart disorders. Read more about these mice in the Cardiovascular Resource Manual ([.pdf] or printed version).

B6.129S4-Timp2tm1Pds/J (008120) These mice are deficient in TIMP-2, tissue inhibitor of metalloproteinase 2, and exhibit locomotor impairment, abnormal fear conditioning behavior, and defective neuronal differentiation. This mutant mouse strain may be used to study extracellular matrix homeostasis, synaptic plasticity in behavior, learning and memory, neuronal differentiation and development of neuromuscular junctions.

B6.C(Cg)-Atcayji/BurJ (008140) Mice homozygous for the spontaneous mutation, jittery (ji) in Atcay (ataxia, cerebellar, Cayman type homolog) exhibit severe, progressive ataxia and die at approximately 4 weeks of age. This strain may be used to study ataxia.

B6.Cg-Tg(THY1-SNCA*A30P)TS2Sud/J (008134) The A30P mutation in this transgenic strain is associated with the development of familial Parkinson's disease. The onset of hind limb mobility problems occurs around 12 months of age (sometimes earlier). It is induced by a loss of motor neurons and associated with the formation of insoluble alpha synuclein aggregates. This strain may be used to study Parkinson's disease.

B6.FVB-Tg(IGFBP2)1Miel/J (008222) This IGFBP-2 transgenic strain may be used to study metabolic homeostasis, adipocyte biology, and the role of insulin-like growth factor binding protein in protecting against obesity- and age-associated complications (such as hypertension and diabetes).

B6;129P2-Dldtm1Ptl/J (008333) Mice homozygous for dihydrolipoamide dehydrogenase or E3 component (Dld) may be used to study early murine embryonic metabolism, including glucose and mitochondrial metabolism during the early post-implantation period. As altered energy metabolism and reductions in alpha-ketoacid dehydrogenase complexes have also been associated with many neurodegenerative disorders, these mice may also be used to stuy related to Parkinson's disease, Huntington's disease, and Alzheimer's disease. Because the metabolic disturbances associated with E3 deficiency (Dld deficiency) cause a variant of Maple Syrup Urine Disease (MSUD) in which there is an accompanying lactic acidosis due to concomitant deficiency of pyruvate dehydrogenase complex, these mice may be useful in such studies or in conjunction with iMSUD mice (Stock No. 006999).

B6.Cg-Tg(CAG-Ngb,-EGFP)1Dgrn/J (007575) Transgenic mice over expressing neuroglobin and GFP in multiple tissues are resistant to experimentally-induced ischemia. When crossed to a mouse model of Alzheimer's disease, the disease phenotype is attenuated. This strain may be used to study cerebral and myocardial ischemia, stroke, and neurodegenerative disease.

B6.FVB(Cg)-Mmp9tm1Tvu/J (007084) Matrix metalloproteinase 9 (Mmp9) mutant mice may be used to study injury response and repair, angiogenesis and inflammatory response.

B6.129-Nrgntm1Kph/J (008233) These neurogranin (Nrgn) deficient mice may be useful for neurological studies involving memory and learning, neuronal signaling pathways (including calmodulin, alpha-CaMKII, protein kinase A, protein kinase C, MAPK, and CREB), attention deficit-hyperactivity disorder (ADHD), and schizophrenia.

B6;129P2-Mecp2tm2Bird/J (006849) Male mice hemizygous for the methyl CpG binding protein 2 (Mecp2) mutation do not breed and develop Rett syndrome symptoms (reduced mobility, hindlimb clasping) at approximately six weeks of age, with death occurring at approximately 11 weeks of age. Heterozygous females are fertile until developing Rett syndrome characteristics at 4-12 months of age. Cre recombinase-mediated removal of the floxed-STOP cassette restores transcription from the targeted allele and MECP2 protein activity to normal, and reverses the Rett syndrome-like neurological defects.

B6.129S1-Car4tm1Sly/J (008217) Carbonic anhydrase 4 (Car4) homozygous knockout mice exhibit decreased sensitivity to an inhibitor, benzolamide, of pH regulation in the extracellular space in the hippocampus when compared to wild-type controls. This mutant mouse strain may be used to study pH shifts that accompany neuronal activity and neuronal physiology.

B6.Cg-Dicer1tm1Bdh/J (006366) These mice contain loxP sites on either side of exon 23. Expression of the targeted allele is indistinguishable from wild-type despite the frt-flanked neomycin cassette. Cre-mediated recombination (resulting in deletion of exon 23) in the germline leads to developmental arrest at embryonic day 7.5 (E7.5). Tissue-specific deletion has been shown to result in loss of microRNA (miRNA) processing. Mutant mice of this strain (or Stock No. 006001) can be used to generate tissue-specific mutants deletions of the endogenous gene for applications in embryonic development, translation, protein processing, and miRNA/siRNA regulation of gene expression.

B6.Cg-Notch1tm2Rko/GridJ (007181) Mice homozygous for this "floxed" Notch1 allele (fN1) are viable and fertile. These mice possess loxP sites on either side of exon 1 of the targeted gene. When bred to mice with a Cre recombinase gene, exon 1 of the targeted gene is deleted in the cre expressing tissue(s). These mice (or Stock No. 006951) may be used to generate tissue-specific mutants to study the development and the role of Notch1 in various tissues.

B6.Cg-Pdgfratm8Sor/EiJ (006492) These mice possess loxP sites on either side of exon 1 and exon 4 of the targeted platelet derived growth factor receptor, alpha polypeptide (Pdgfra) gene. When used in conjunction with a Cre recombinase-expressing strain, this strain is useful in generating tissue-specific mutants of the floxed allele.

129S1/Sv-Bchetm1Loc/J (008077); B6.129S1-Bchetm1Loc/J (008087) These butyrylcholinesterase (Bche) mutant mice are a model for human butyrylcholinesterase deficiency and may be used to study metabolic effects of organophosphorus toxicants or nerve agents, neurotransmitter function, and anti-Alzheimer's drug therapies.

B6.129P2-Sorl1Gt(pGT1tfr)1Ucd/J (007999) Mice homozygous for this gene trapped allele exhibit a higher incidence of diffuse immunoreactive beta-amyloid deposits when compared to wild-type controls. This mutant mouse strain may be useful in studies of Alzheimer's disease.

B6;129-Sncatm1Sud Sncbtm1.1Sud/J (006390) In homozygous mice, no protein product from these targeted genes is detected in brain tissue. Dopamine levels in the double targeted mice are decreased by approximately 20 percent (compared with single mutants or wildtype controls), but dopamine uptake and release from isolated nerve terminals is normal. Serotonin levels in double mutants are unchanged from that of wild-type. This mutant mouse strain represents a model that may be useful in studies of synaptic function and neurodegenerative disease.

B6.Cg-Tg(ACTB-Ub*G76V/GFP)1Dant/J (008111); B6.Cg-Tg(ACTB-Ub*G76V/GFP)2Dant/J (008112) Both ubiquitin/proteasome system reporter lines, UbG76V-GFP/1 (Stock No. 008111) and UbG76V-GFP/2 (Stock No. 008112), may be useful for monitoring the role of ubiquitin/proteasome-dependent proteolysis in diverse disorders, and for efficacy trials testing the effect of compounds on the ubiquitin/proteasome system (including the use of proteasome inhibitors as novel anticancer drugs).

B6.Cg-Tg(Camk2a-Crebbp*)1364Tabe/J (007574) These CaMKII?-FLAG-CBP?1 transgenic mice may be useful for behavioral and learning neuroscience research including hippocampal synaptic plasticity, spatial/contextual hippocampus-dependent memory, and cognitive endophenotypes of Rubinstein-Taybi Syndrome (RTS).

Researchers now have six new mouse model list PDFs to refer to when selecting mice for neurobiology research.

Alzheimer's disease [.pdf]
Huntington's disease [.pdf]
Neurobiology [.pdf]
Parkinson's disease [.pdf]
ALS/Spinal muscular atrophy [.pdf]
Research tools for neurobiology [.pdf]

B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J (007708) In this strain, the neuropathogenic polyQ-mutant variant of the human Huntingtin protein (mhtt-exon1; 103Q) is inserted into a gene trap ROSA 26, Philippe Soriano (Gt(ROSA)26Sor) transgene. Expression of mhtt-exon1 is blocked by an upstream loxP-flanked trasncriptional STOP sequence. When this strain is bred to one with a Cre recombinase gene under the control of a promoter of interest, offspring are produced in which the STOP sequence is deleted in the tissue of interest, allowing mhtt-exon1 to be expressed. This strain may be used to research Huntington's disease or other polyQ disorders.

B6.Cg-Tg(tetO-APPSwInd)885Dbo/J (007049) Hemizygotes for this tet-APPswe/ind transgene are viable and fertile. They express a form of chimeric mouse/human amyloid precursor protein (APP695) bearing the Swedish (KM570/571NL) and Indiana (V617F) APP mutations associated with Alzheimer's disease (APP695swe/ind) under the control of a tetracycline-responsive promoter element (TRE or tetO). This strain may be used to research Alzheimer's disease and other neurodegenerative autopathies.

B6.Cg-Tg(Thy1-ChR2/EYFP)18Gfng/J (007612) This strain expresses the light-activated ion channel, Channelrhodopsin-2 (from the green alga Chlamydomonas reinhardtii), and yellow fluorescent protein fusion gene (ChR2-YFP) under the control of the mouse thymus cell antigen 1 promoter. Hemizygotes are viable, fertile, and look and act normally. These strains may be used to research neural activity by light stimulation.

B6.Cg-Tg(Thy1-ChR2/EYFP)9Gfng/J (007615) This strain expresses the light-activated ion channel, Channelrhodopsin-2 (from the green alga Chlamydomonas reinhardtii), and yellow fluorescent protein fusion gene (ChR2-YFP) under the control of the mouse thymus cell antigen 1 promoter. Hemizygotes are viable, fertile, and look and act normally. These strains may be used to research neural activity by light stimulation.

B6.Cg-Tg(Thy1-cre/ESR1,-EYFP)AGfng/J (007606) This "SLICK" (single-neuron labeling with inducible Cre-mediated knock-out) strain has a tamoxifen- inducible cre-mediated recombination system and enhanced yellow fluorescent protein (YFP) driven by two bidirectional copies of the mouse thymus cell antigen 1 promoter. When this strain is mated with one containing a loxP site flanked sequence of interest, their offspring may be used to generate tamoxifen-induced, cre-mediated targeted neuron-specific deletions in neurons where the Thy1 promoter is active. These strains may be used in neurobiology research.

B6.Cg-Tg(Thy1-cre/ESR1,-EYFP)VGfng/J (007610) This "SLICK" (single-neuron labeling with inducible Cre-mediated knock-out) strain has a tamoxifen- inducible cre-mediated recombination system and enhanced yellow fluorescent protein (YFP) driven by two bidirectional copies of the mouse thymus cell antigen 1 promoter. When this strain is mated with one containing a loxP site flanked sequence of interest, their offspring may be used to generate tamoxifen-induced, cre-mediated targeted neuron-specific deletions in neurons where the Thy1 promoter is active. These strains may be used in neurobiology research.

129-Braftm1Sva/J (006373) Homozygotes for this floxed Braf transforming (Braf) gene are viable and fertile. When bred to mice expressing Cre recombinase under the control of a promoter of interest, they produce offspring in which Braf exon 12 is deleted in the tissue of interest. This strain may be used to research Ras/Raf and MEK/ERK signaling, synaptic (neural) plasticity, learning, and memory.

B6;129P2-Mecp2tm1Bird/J (006847) This strain posseses two functional loxP sites flanking exons 3-4 of the methyl CpG binding protein 2 (Mecp2) gene on the X chromosome. When bred to a strain expressing Cre-recombinase, offspring are produced in which exons 3-4 of the gene are deleted in the cre-expressing tissue(s). This strain may be used to research Rett syndrome.

B6.129P2-Mecp2tm2Bird/J (006849) This strain possesses a loxP-flanked STOP cassette in intron 2 of the methyl CpG binding protein 2 (Mecp2) gene on the X chromosome. Hemizygous males do not breed and develop Rett syndrome symptoms around 6 weeks of age and die around 11 weeks of age. Hemizygous females are fertile until they develop Rett syndrome characteristics between 4-12 months of age. Cre-mediated removal of the floxed-STOP cassette reverses Rett syndrome defects. This strain may be used to research Rett syndrome.

B6.129P2-Vamp2tm1Sud/J (006380) Whereas heterozygotes for this vesicle-associated membrane protein 2 (Vamp2) gene knockout appear normal, newborn homozygotes have a shoulder hump that may be caused by excess brown fat and die shortly after birth. Spontaneous synaptic vesicle fusion and fusion induced by hypertonic sucrose are decreased approximately 10-fold, and fast Ca2+-triggered fusion is decreased more than 100-fold. This strain may be used to research synaptic fusion mechanisms.

JAX Mice strain B6CBA-Tg(HDexon1)62Gpb/1J (002810) Huntington disease model.

B6SJL-Tg(APPSwFlLon,PSEN1*M146L*L286V)6799Vas/J (006554) These transgenic mice (called 5XFAD) co-overexpress human APP(695) with the Swedish (K670N, M671L), Florida (I716V), and London (V717I) Familial Alzheimer's Disease (FAD) mutations in the same APP molecule, as well as a human PS1 harboring two FAD mutations, M146L and L286V, each using the neuron-specific elements of the mouse Thy1 promoter to drive overexpression in the brain. Hemizygous mice are viable and fertile. They rapidly recapitulate major features of Alzheimer's disease amyloid pathology and may be useful models of intraneuronal Aβ42 induced neurodegeneration and amyloid plaque formation.

B6.Cg-Tnks2tm1.1Yjc/J (006200) Homozygotes for this targeted mutation of the tankyrase, TRF1-interacting ankyrin-related ADP-ribose polymerase 2 (Tnks2) gene are viable and fertile but are significantly underweight. They may be used to research telomerase function and developmental and metabolic pathways.

New JAX® Mice Strains under Development
Posted 15 January 2007

B6;129-Rab3btm1Sud, Rab3atm1Sud, Rab3dtm1Rja, Rab3ctm1Sud/J (006375) Homozygotes for all four of these RAS oncogene targeted mutations die from respiratory problems shortly after birth. Except for the loss of rabphilin, synapse structure and brain composition are normal. Excitatory postsynaptic currents (EPSCs) induced by action potentials are ~30% smaller than they are in controls. This strain may be used to research synaptic vesicle exocytosis mechanisms in neurons.

B6.129S4-Abcb7tm1Mdf/J (006490) Homozygotes for this targeted mutation of the ATP-binding cassette, sub-family B (MDR/TAP), member 7 (Abcb7) gene are viable, fertile, and have no reported neurological or hematological abnormalities. In offspring of this strain mated with one expressing cre recombinase in tissues of interest, exons 9 and 10 of the Abcb7 gene are deleted. This strain may be used to research cytosolic Fe-S cluster assembly and metabolism, Friedreich ataxia, anemia, and hematopoiesis.

B6.129-Skiltm2Spw/J (006497) Homozygotes for the "Snoex1" mutation (a targeted mutation of the SKI-like (Skil) gene) are viable, fertile, and have no reported gross morphological defects. They have T cell proliferation and activation defects, increased sensitivity to TGFB, and altered growth of cultured mouse embryo fibroblasts (MEFs). They may be used to research T cell activation, T cell receptor stimulation, and TGFB signaling.

STOCK Igh-4tm1.1Ksho/J (006052) Homozygotes for this targeted mutation of the immunoglobulin heavy constant gamma (Igh-4) gene are viable and fertile. They and heterozygotes have impaired primary and secondary IgG1 responses. This strain may be used to research the role of IgG1 immunoglobulins in immune responses.

New JAX® Mice Strains under Development
Posted 6 December 2006

B6.129-Btlatm1Kmm/J (006353), C.129-Btlatm1Kmm/J (006339), and NOD.129- Btlatm1Kmm/J (006355) Homozygotes for this targeted mutation of the B and T lymphocyte associated (Btla) gene are viable, fertile, and look and behave normally. They are abnormally sensitive to antigen-induced experimental autoimmune encephalomyelitis (EAE, human model of multiple sclerosis). They may be used to research immune response, autoimmunity, and transplantation.

B6;129S4-Apoa2tm1Bres/J (006258) Homozygotes for this targeted mutation of the apolipoprotein A-II (Apoa2) gene are viable and fertile. Whether fasted or unfasted, they have decreased plasma HDL cholesterol and free fatty acid levels, and, when fasted, decreased plasma glucose and insulin levels. They may be used to research lipid metabolism, atherosclerosis, heart disease, insulin resistance, and diabetes.

B6;129S4-Apoa4tm1Bres/J (006404) Homozygotes for this targeted mutation of the apolipoprotein A-IV (Apoa4) gene are viable and fertile. They have decreased plasma levels of cholesterol, free fatty acids, high density lipoprotein (HDL), and very low density lipoprotein (VLDL). They may be used to research lipid metabolism, atherosclerosis, heart disease, appetite regulation, intestinal lipid absorption, and inflammatory bowel disease.

B6;129S4-Thbs2tm1Bst/J (006238) Homozygotes for this targeted mutation of the thrombospondin 2 (Thbs2) gene are viable and fertile. They have numerous skin, skeletal, and blood disorders. They may be used to research collagen fibrillogenesis in skin and tendon, angiogenesis and vascular pathophysiology, wound healing, chemically-induced tumor progression, and Ehlers Danlos syndrome.

B6;CBA-Tg(ACTB-lacZ-WGA)330Bbm/J (006465) These transgenic mice constitutively express lacZ under the control of the CMV enhancer/chicken actin promoter. Homozygotes are viable, fertile, and look and behave normally. They can be crossed with a Cre recombinase-expressing strain to produce offspring in which the lack of reporter (lacZ) expression can be distinguished from a lack of Cre recombinase expression and in which Cre excision activity of individual cells can be assessed. This strain may be used to trace transneuronal or trans-synaptic connections and circuits in the brain and spinal cord.

B6.Cg-Gusbmps/BrkJ (006407) Homozygotes for this mucopolysaccharidosis type VII or MPS VII (mps) mutation of the glucuronidase, beta (Gusb) gene do not express beta glucuronidase. They are viable, but females have a lactation defect. Both sexes are deaf, have skeletal and connective tissue anomalies, are unsuccessful breeders, have short thick long bones, "pug type" noses, hepatomegaly, splenomegaly, and corneal clouding. They are a model of Sly Disease in humans and may be used to develop therapies for lysosomal storage diseases.

C57BL/6J-Tg(ACTB-NOTCH1)1Shn/J (006481) Mice of this transgenic strain are viable, fertile, and behave normally. The expression of the intracellular domain of the human notch gene homolog 1 (NOTCH1) transgene is prevented by a "Lox-STOP-Lox" cassette. When this strain is bred to a Cre-expressing strain, the "floxed stop" region in the resulting offspring is excised, and human NOTCH1 is expressed in cre-expressing tissue(s). This strain may be used to research early neural progenitor cell development and apoptosis, and for tissue specific Notch activation.

NOD.Cg-Rag1tm1Mom H2-Ab1tm1Gru Tg(CD2-CD4,HLA-DQA1,HLA-DQB1)1Ell/EllJ (006022) This strain is deficient in the recombination activating gene 1(Rag1) and the histocompatibility 2, class II antigen A, beta 1 (H2-Ab1) genes, and is transgenic for the human CD2-CD4 and HLA-DQ8 genes. It lacks T and B cells, is resistant to diabetes, and does not develop cardiomyopathy. This strain may be used in adoptive transfer experiments and to understand the role of MHC class II in autoimmunity.

New Strains Available from Jackson Labs
Posted 9 April 2006

B6.129S4-Ucp2tm1Lowl/J (005934) Homozygotes for this knock-out of the uncoupling protein 2 (mitochondrial, proton carrier) (Ucp2) gene are viable and fertile. They have 18% less blood glucose, and, whether fasted or fed, have approximately three times more serum insulin than do wild-type controls. Mitochondria isolated from their dopaminergic mesencephalic nigral cells have more reactive oxygen species, fewer mitochondria, and a greater sensitivity to MPTP (mimicking Parkinson's disease) than do wild-type controls. This mutant may be useful for researching diabetes, glucose-dependent metabolism-secretion coupling, aerobic respiration, Parkinson’s disease, epilepsy, stroke, and other neurodegenerative diseases.

B6.129P-Psen2tm1Bdes/J (005617) Homozygotes for this targeted mutation of the presenilin 2 (Psen2) gene are viable, fertile, and look and behave normally. By the time they are three months old, their alveoli have thickened walls, fibrotic deposits, and hemorahages. This mutant may be useful for researching Alzheimer's disease and vascular defects.

B6;129P2-Bace2tm1Bdes/J (005618) Homozygotes for this targeted mutation of the beta-site APP-cleaving enzyme 2 (Bace2) gene are viable, fertile, and look and behave normally. Proteins generated from the targeted locus lack beta-secretase activity. This mutant may be useful for researching Alzheimer's disease.

C57BL/6-Tg(tetO-CDK5R1/GFP)337Lht/J (005706) Hemizygotes for this cyclin-dependent kinase 5, regulatory subunit 1 (Human) transgene are viable, fertile, and look and behave normally. The transgene can be inducibly expressed in offspring produced by breeding this mouse with mice having the tetracycline-controlled transactivator protein (tTA). Homozygotes for the transgene may not be viable. Hemizygotes may be useful for researching Alzheimer's disease and other neurodegenerative tauopathies, amyotrophic lateral sclerosis (ALS), Niemann Pick Type C (NPC) disease, and Parkinson's disease.

New Strains Available from Jackson Labs
Posted 11 February 2006

B6.FVBTg(ITGAMDTR/EGFP)34Lan/J (Stock No. 006000) These transgenic mice have a diphtheria toxin (DT) inducible system that transiently depletes macrophages. Homozygotes look and behave normally . The transgene contains a fusion product involving simian diphtheria toxin receptor and green fluorescent protein under the control of the human ITGAM (integrin alpha M) promoter (CD11b). Intraperitoneal injection of DT ablates monocyte/macrophage cells (but not polymorphonuclear cells) in the peritoneal cavity, kidney, and ovaries within 12 hours. Macrophage populations are restored four days later. This strain may be useful for researching the specific role of macrophages in the immune response.

C57BL/6J-mt A/J /NaJ (Stock No. 005545) This mitochondrial substitution or conplastic strain has the C57BL/6J mitochondrial DNA replaced by that of the A/J strain. The two strains differ in their susceptibility to diseases such as arthritis, asthma, atherosclerosis, cancer, several infectious diseases, inflammatory responses, and physiological, behavioral and sensory phenotypes. Using this strain may accelerate identifying and mapping quantitative trait loci (QTLs) associated with these diseases.

B6.129S-Galr1tm1Tpi /J (Stock No. 005695) Mice homozygous for this knock-out of the galanin receptor 1 (Galr1) gene are viable, fertile, normal-sized, and look normal. The donating investigator indicates that they do not exhibit seizure behavior sometimes seen in incipient congenic mice with a mutation of the same gene. They can be used to research receptor defects and various other neurobiological phenotypes.

D1Lac.Cg-Tg(Tcra,Tcrb)24Efro/J (Stock No. 005694) These transgenics for a fully functional bovine and mouse type II collagen (260-267) specific IAq-restricted T cell receptor are viable, fertile, and normal-sized. The transgene confers severe accelerated autoimmune arthritis following type II collagen (CII) immunization. The splenocytes from tolerized mutants have altered activation and memory cell surface markers and secrete higher levels of Th2 cytokines than do controls. Estradiol completely prevents collagen-induced arthritis (CIA). This mutant may be useful for researching CIA, rheumatoid arthritis, T regulatory cells, and may also serve as a source of T cells that mediate the development of autoimmune diseases.

Are you interested in obtaining older C57BL/6J mice? You can order 8 and 15 week-old mice. Researchers may also register to receive retired breeders (aged 7-8 months).

B6.129S4-Grin1tm2Stl/J (Stock No. 005246)
These mice possess loxP sites that flank approximately 12kb of the glutamate receptor, ionotropic, NMDA1 (zeta 1) (Grin1, commonly called NMDAR1) gene. The flanked sequence encodes the entire transmembrane domain and C-terminal region of the Grin1 gene. Homozygotes are viable, fertile, and look and behave normally. When used in conjunction with a Cre recombinase-expressing strain, this strain is useful for generating tissue-specific mutants of the floxed Grin1tm2Stl allele.

129S6/SvEvTac-Car3tm1Gkim/J (Stock No. 005358)
Homozygotes for this knock-out of the carbonic anhydrase 3 (Car3) gene are viable, fertile, and look and behave normally. Their tissues and the distribution of fatty-acids in their serum, muscle, liver, and fat tissues are comparable to those of wild-type mice. Their soleus muscles have shorter relaxation and half-relaxation times for single and tetanic twitches and a slightly smaller tetanic force than do the same muscles in wild-type mice. They express about 30% more carbonic anhydrase 13 than do wild-type mice. This strain may be used for neurobiology and metabolism research.

B6;129-Pax3f^tm1(cre)Joe/J (Stock No. 005549)
This strain expresses Cre recombinase from the endogenous Pax3 gene locus. Homozygotes fail to develop past embryonic day (E) 18.5. At E13.5, they have severe cardiac and neural tube (exencephaly) defects, missing limb musculature, and reduced or missing dorsal root ganglia. No endogenous Pax3 gene product (protein) is detected in homozygotes and approximately one half of the endogenous gene product (protein) is detected in heterozygotes by Western blot analysis.Heterozygotes are viable, fertile, behave normally, and look normal, except that about half of them have a white belly spot. This strain may be used to research development and to map the lineage of neural crest and somite derivatives.

B6;D2-Tg(S100B-EYFP)1Wjt/J (Stock No. 005620)
Homozygotes for the S100B-EYFP transgenic insert are viable, fertile, and look and behave normally. They express Enhanced Yellow Fluorescent Protein (EYFP) under the direction of the human S100 calcium-binding protein, beta (S100B) (neural) promoter. The Schwann cells of their sternomastoid, extensor digitorum longus, triangularis sterni, and diaphragm muscles and the motor axons of their triangularis sterni fluoresce strongly, as do their glial cells at birth and their motor axons at postnatal day 7. They may be used for vital imaging of neuronal and glial cells.

B6;D2-Tg(S100B-EGFP)1Wjt/J (Stock No. 005621)
Homozygotes for the S100B-EGFP transgenic insert are viable, fertile, and look and behave normally. They express Enhanced Green Fluorescent Protein (EGFP) under the direction of the human S100 calcium-binding protein, beta (S100B) (neural) promoter. The Schwann cells of their sternomastoid, soleus, extensor digitorum longus, triangularis sterni and diaphragm muscles, some astrocytes, Bergmann glia, and a few muscle macrophages fluoresce strongly. GFP expression is detected at birth. This strain may be useful for vital imaging of neuronal cells, glial cells, and neuromuscular junctions.

B6.Cg-Tg(Camk2a-cre)T29-1Stl/J (Stock No. 005359)
Homozygotes for the Camk2a-cre transgenic insert are viable, fertile, and look and behave normally. They express Cre recombinase in the forebrain, specifically in the CA1 pyramidal cell layer of the hippocampus, under the control of the mouse calcium/calmodulin-dependent protein kinase II alpha (Camk2a) gene promoter. They may be used for neurobiology research.

B6.129S7-Trp63tm1Brd/J (Stock No. 003568)
Homozygotes for this targeted mutation of the transformation related protein 63 (Trp63) gene are born alive but die several hours after birth. They have striking developmental defects, including truncated forelimbs, missing hindlimbs, transparent skin, and no hair follicles. Their internal organs seem normal. They lose 30 times more water than normal and appear to die from dehydration. Heterozygotes are viable but exhibit a shortened life span (95weeks) and display features of accelerated aging. These mice may be useful in studies related to cellular senescence and aging.

New Model for Engraftment with Human Hematopoietic Stem Cells
A new mouse model that can support a human immune system was recently developed by The Jackson Laboratory's Dr. Leonard Shultz and collaborators. The mouse will allow scientists to 1) perform the critical studies necessary to improve hematopoietic stem cell (HSC) transplants for treating leukemia, sickle cell disease, and other blood disorders (all without putting patients at risk), and 2) study the HIV(AIDS) virus in a model that mimics the human immune system better than does any previously constructed model. (Strain Name: NOD.Cg-Prkdc^scidIl2rg^tm1Wjl/SzJ; Stock Number: 005557

*Note: We are also developing an aging colony for STOCK Mapttm1(GFP)Klt Tg(MAPT)8cPdav/J (stock number 004808). If you would like advance notification of strain availability and the opportunity to order prior to aged animals being published as available, please register interest at our web site.

If you are interested in obtaining aged mice, contact JAX® Services at: 1-800-422-MICE (6423). Orders for small numbers of aged mice will be filled on a first-come, first-serve basis for as long as the mice are available. Pricing for these mice is determined by their age (contact JAX® Services for more information). License and use restrictions for some of these mice may apply. See the strain data sheets for details.

STOCK Tg(Tek-rTA,TRE-lacZ)1425Tpr/J (Stock No. 005493) These mice harbor co-injected transgenic constructs: 1) the reverse tetracycline-controlled transactivator (rtTA) protein expressed under the direction of the endothelial-specific receptor tyrosine kinase enhancer/promoter (Tek) and 2) a nuclear-localizing beta-galactosidase gene under the control of a tetracycline-responsive element (TRE). The model allows the inducible expression of genes in the vascular endothelium during early development and adulthood.

FVB-Tg(ITGAM-DTR/EGFP)34Lan/J (Stock No. 005515) These mice have a diphtheria toxin (DT) inducible system that transiently depletes macrophages. The transgene insert contains a fusion product involving simian diphtheria toxin receptor and green fluorescent protein under the control of the human ITGAM (integrin alpha M) promoter (CD11b). The model may be useful for researching the role of macrophages in the immune response.

B6.129S-Sca1tm1Hzo/J (Stock No. 005601) These mice carry a knock-in of the spinocerebellar ataxia 1 homolog gene. Heterozygotes are viable but have a reduced lifespan (34-40 weeks). They mimic many of the symptoms of spinaocerebellar ataxia type 1 (SCA1) and may be used for researching it and other neurodegenerative diseases.

NOD.B6-Tg(HLA-A2.1)1Enge/DvsJ (Stock No. 005512) Homozygotes for the (HLA-A2.1)1Enge transgene ubiquitously express significant quantities of the human MHC class I HLA-A2.1 molecules, resulting in a significantly accelerated rate of diabetes onset in either the hemizygote or homozygote transgenic mouse when compared to NOD/Lt controls. They may be used to research the role of MHC class I molecules in type 1 diabetes.

Down Syndrome Model Distribution Expanded
http://jaxmice.jax.org/strain/001924.html The most studied mouse model for Down Syndrome is the Ts(17)65Dn (abbreviated Ts65Dn) mouse (Stock Number 001924). This model was developed by Dr. Muriel Davisson's research group at The Jackson Laboratory with funding from the National Institute of Child Health and Human Development (NICHD); it was first published in 1990. The Ts65Dn stock is maintained in and distributed from The Jackson Laboratory's Cytogenetic Models Resource, which also is supported by the NICHD (N01 HD73265). The NICHD has recently increased funding to expand the Ts65Dn colony, which will make it more available, and maintain it at a high health status.

Mouse Models for Alzheimer's Research: Winter 2005
See Most Recent List of AD Mouse Models (.pdf)

Aged Alzheimer's Disease Models
One of the hallmarks of Alzheimer's Disease (AD) is the presence of extracellular amyloid deposits in the brain, most often observed in the tissues of the cortex, hippocampus, and amygdala. A co-integrated double transgenic mouse, B6C3-Tg(APPswe,PSEN1dE9)85Dbo/J (Stock Number 004462), exhibits amyloid deposits at 6 to 7 months age.

Additional Aged Alzheimer's Disease Models
The Alzheimer's Disease Mouse Models Resource (ADMMR) has recently acquired several additional mouse models that develop AD related characteristics as they age. These include B6.Cg-Tg(PDGFB-APPSwInd)20Lms/J (or "J20", Stock Number 004661) that express a mutant form of human APP bearing both the Swedish (K670N/M671L) and the Indiana (V717F) mutations and its wildtype control strain, B6.Cg-Tg(PDGFB-APP)5Lms/J (or "I5"; Stock Number 004662), as well as STOCK Mapttm1(GFP)Klt Tg(MAPT)8cPdav/J (or "hTau"; Stock Number 004808). Orders for breeding units, or small numbers of breeding age mice may be placed through our Customer Service Department.

If you are interested in obtaining aged mice, a dedicated colony will be established based on demand. Please register your interest in obtaining aged mice from the following strains below:

Other Jackson Lab Updates: Newly Available Strains

Selective Neuronal Loss in Weeble Mutant Mice
Research Applications:

Neurobiology Research: Ataxia (Movement) Defects
Neurobiology Research: Cerebellar Defects
Neurobiology Research: Epilepsy

Gene Symbol:Inpp4awbl
Gene Name: weeble

JAX® Mice Strain Name: CByJ.B6-Inpp4awbl/FrkJ
Stock Number: 004913
Reference: Nystuen et al., 2001

New Model for Human Lysomal Storage Disease
Research Applications:

• Developmental Biology Research: Growth Defects (homozygous)
• Developmental Biology Research: Skeletal Defects
• Immunology and Inflammation Research: Immunodeficiency and Autoimmunity (B & T cell deficiency)
• Internal/Organ Research: Lymphoid Tissue Defects (B & T cell deficiency)
• Metabolism Research
• Mouse/Human Gene Homologs: mucopolysaccharidosis type VII, GUSB deficiency
• Neurobiology Research: Behavioral and Learning Defects
• Research Tools: Cancer Research (B & T cell deficiency) (xenograft/transplant host)
• Research Tools: Toxicology Research (xenograft/transplant host)
• Virology Research: B & T Cell Deficiency (AIDS research tool)
• Sensorineural Research

Gene Symbol: Prkdcscid Gusmps
Gene Names: severe combined immune deficiency; beta glucuronidase, mucopolysaccharidosis VII

JAX® Mice Strain Name: NOD.Cg-Prkdcscid-Gusmps/SndsJ
Stock Number: 005053
Reference: Hofling et al., 2003

More Spinal Muscular Atrophy Mouse Models Under Development

FVB.Cg-Tg(SMN2*delta7)4299Ahmb Tg(SMN2)89Ahmb Smntm1Msd/J (Stock Number 005025)
FVB.Cg-Tg(SMN2)89Ahmb Tg(SMN1*A2G)2023Ahmb Smntm1Msd/J (Stock Number 005026)
FVB.Cg-Tg(SMN2)89Ahmb Smntm1Msd/J (Stock Number 005024)

Molecular Basis for Memory and Learning
Research Applications:

Developmental Biology Research: Embyronic Lethality (Homozygous) (incomplete);
• Developmental Biology Research: Internal/Organ Defects (ovary; uterus);
• Neurobiology Research: Behavioral and Learning Defects;
• Internal/Organ Research: Other Organ Defects;
• Reproductive Biology Research: Developmental Defects Affecting Gonads (germ cell deficient);
• Reproductive Biology Research: Fertility Defects

Gene Symbols: Creb1/Esr1
Gene Names: cAMP responsive element binding protein 1 repressor/estrogen ligand-binding domain fusion protein
Gene Common Names: Esr1: ER[a]; Era; Eralpha; ESR; Estr; Estra; Nr3a1 Creb1: Creb; Creb-1

JAX® Mice Strain Name: C3H-Tg(Camk2a-Creb1/Esr1)3Sva/J
Strain Common Name: CREB-IR
Stock Number: 004995
Reference: Kida et al., 2002

The Role of Oxidative Stress in Diabetes
Research Applications:

• Metabolism Research
• Neurobiology Research: Metabolic Defects
• Neurobiology Research: Neurodegeneration

JAX® Mice Strain Name: NOD.FVB-Tg(INS-SOD2)3Pne/PneJ
Strain Common Name: IsNOD NOD.MnSOD
Stock Number: 005113

Cardiovascular Research: Hypercholesterolemia

Autoimmune Disease Studies
Research Applications:

Cancer Research: Growth Factors/Receptors/Cytokines
• Diabetes and Obesity Research: Type 1 Diabetes (IDDM) Analysis Strains (NOD Congenics with Mutations Affecting Cytokine Production by Autoreactive T Cells)
• Immunology and Inflammation Research: CD Antigens, Antigen Receptors, and Histocompatibility Markers; Growth Factors/Receptors/Cytokines

JAX Mice
Strain Name: NOD.129(B6)-Ctla4tm1All/DoiJ
Strain Common Names: NOD.CD-152; NOD.Cd152; NOD.Ctla-4; NOD.Ly-56
Stock Number: 005144
Reference: Luhder et al., 2000

Cre-Induced Constituitive Hedgehog Signaling
Research Applications:

• Developmental Biology Research
• Embryonic Lethality (Homozygous)
• Internal/Organ Defects (heart); Neural Tube Defects

Diabetes and Obesity Research: Type 1 Diabetes (IDDM)

• Cancer Research
• Growth Factors/Receptors/Cytokines
• Immunology and Inflammation Research
Growth Factors/Receptors/Cytokines

Amyotrophic Lateral Sclerosis Studies
Research Applications:

• Metabolism Research
• Neurobiology Research
• Metabolic Defects
• Neurobiology Research
• Neurodegeneration

Spinal Muscular Atrophy Mouse Model
Research Applications:
Neurobiology Research: Ataxia (Movement) Defects (Spinocerebellar (ataxia), Neurodegeneration, Neuromuscular Defects
Gene Symbols: SMN2; Smn
Gene Names: survival of motor neuron 2, centromeric, human ; survival motor neuron
Gene Common Names: Smn: Gemin1, SMN1
JAX® Mice Strain Name: FVB.Cg-Tg(SMN2)1Hung Smntm1Hung/J
Strain Common Name: SMA-like mice line 2
Stock Number: 005058
Jax Datasheet

Macrophage & Dendritic Cell Fluorescence/Depletion
Research Applications:
Research Tools: Apoptosis Research; Cell Biology Research; Fluorescent Proteins; Hematological Research; Immunology and Inflammation Research
Gene Symbol: GFP; NGFR/FKBP12
Gene Name: Green Fluorescent Protein; NGFR (deltaLNGFR, low affinity nerve growth factor receptor, human)/FKBP12 (FK506 binding protein 1A, 12kDa)
Strain Name: C57BL/6J-Tg(Csf1r-GFP, NGFR/FKBP12)2Bck/J
Strain Common Name: MAFIA mouse
Stock Number: 005070
Jax Datasheet

Transgenic Mice Expressing a Mutant Form of the Human Amyloid Precursor Protein
Strain Name: B6.Cg-Tg(PDGFB-APPSwInd)20Lms/J
Strain Common Names: J20 line; PDGF-APPSwInd
Stock Number: 004661
Gene Symbol: APP
Gene Name: amyloid beta (A4) precursor protein (protease nexin-II, Alzheimer disease)
Status: Newly Available
Jax Datasheet

Transgenic Mice Expressing Wildtype Human Amyloid Precursor Protein
Strain Name: B6.Cg-Tg(PDGFB-APP)5Lms/J
Strain Common Names: I5 line, wildtype control for J20 line; PDGF-APPWT
Stock Number: 004662
Gene Symbol: APP
Gene Name: amyloid beta (A4) precursor protein (protease nexin-II, Alzheimer disease)
Status: Newly Available
Jax Datasheet

Presenilin 1 Knockout Mouse Model
Strain Name: B6.129-Psen1tm1Shn/J
Stock Number: 003615
Gene Symbol: Psen1
Gene Name: presenilin 1
Status: Level 4 - Up to 3 pairs or 6 individual mice can be shipped per order; 1 order at a time. Expected delivery for most strains is 1 to 3 months. Call to inquire about ordering greater quantities
Jax Datasheet

Mouse Model for Alzheimer's Disease Pathogenesis Studies
Strain Name: STOCK Mapttm1(GFP)Klt Tg(MAPT)8cPdav/J
Strain Common Names: htau mice
Stock Number: 004808
Gene Symbol: MAPT; Mapt; GFP
Gene Name: microtubule-associated protein tau
Status: Under Development. Estimated Available for Sale Date: July 2004. Please register interest.
Jax Datasheet

be published or accepted for publication in a peer-reviewed journal

have generated significant demand for distribution from the research community

be made available for broad distribution, at least to non-profit research institutions, according to the NIH Sharing and Distributing Mouse Resources guidelines.

Posted 3 November 2003

Three Alzheimer's Disease transgenic mouse models with targeted mutations are now available or under development at The Jackson Laboratory.

1. Alzheimer's Disease-Related Strain

Strain Name: B6.Cg-Tg(PDGFB-APPSwInd)20Lms
Common Names: J20 line; PDGF-APPSwInd
Stock Number: 004661
Gene Symbol: APP
Gene Name: amyloid β (A4) precursor protein (protease nexin-II, Alzheimer disease)
Common Names: AAA; ABETA; AD1; Amyloid beta (A4) precursor protein; CVAP; amyloid β-peptide
Standard Supply: Under Development, please register interest

Key Phenotypic Components

These transgenic mice express a mutant form of the human amyloid protein precursor bearing both the Swedish(670 K-N/671M-L) and the Indiana (717V->F) mutations (APPSwInd).
Expression of the transgenic insert is directed by the human platelet-derived growth factor β polypeptide (PDGFB) promoter.
Hemizygotes express immunodetectable transgene product in cerebral neurons, with the highest level of expression occurring in the neocortex and hippocampus.
Enzyme-linked immunosorbent assay (ELISA) analysis reveals approximate total amyloid β peptides (Aβ) and 42 amino acid length A β in neocortical and hippocampal tissue from mutant mice.
At 5 to 7 months of age diffuse Aβ deposition in the dendate gyrus and neocortex forms.
Amyloid deposition is progressive with all transgenic mice exhibiting plaques by age 8 to 10 months.

For more information, see the data sheet for this strain.

2. Control for Stock Number 004661 (Above Strain)

Strain Name: B6.Cg-Tg(PDGFB-APP)5Lms
Common Names: I5 line, wildtype control for J20 line; PDGF-APPWT
Stock Number: 004662
Gene Symbol: APP
Gene Name: amyloid β (A4) precursor protein (protease nexin-II, Alzheimer disease)
Standard Supply: Under Development, please register interest

Key Phenotypic Components

These transgenic mice express a wildtype human amyloid protein precursor (APP) under the control of the human platelet-derived growth factor beta polypeptide (PDGFB) promoter.
Hemizygotes express immunodetectable transgene product in cerebral neurons, with the highest level of expression occurring in the neocortex and hippocampus.
Enzyme-linked immunosorbent assay (ELISA) analysis of neocortical and hippocampal tissue reveals approximate total amyloid beta peptides (A β) levels and 42 amino acid length A β levels that are lower than levels found in the APPSwInd mutant line.
No amyloid plaques are detected by immunohistochemistry at 8-10 months of age or at 24 months of age.
Mutants display age dependent decrease in density of synaptophysin-immunoreactive presynaptic terminals indicative of neurodegeneration.

Primary Reference
Mucke L, Masliah E, Yu GQ, Mallory M, Rockenstein EM, Tatsuno G, Hu K, Kholodenko D, Johnson-Wood K, McConlogue L. 2000. High-level neuronal expression of aβ 1-42 in wild-type human amyloid protein precursor transgenic mice: synaptotoxicity without plaque formation. J Neurosci 20:4050-8. Abstract

For more information, see the data sheet for this strain.

3. Alzheimer's Disease-Related Strain

Strain Name: B6C3-Tg(APP695)85Dbo Tg(PSEN1)85Dbo
Common Name: Mo/Hu APPswe PS1dE9
Stock Number: 004462
Gene Symbols: APP695; PSEN1
Gene Names: amyloid β (A4) precursor protein (chimeric); presenilin 1 (Human)
Common Names for APP695: APP; APP PDGFB; FAD; amyloid; chimeric mouse/human beta-amyloid precursor protein
Common Names for PSEN1: AD3, presenilin 1 (Alzheimer disease 3); FAD; PS1; S182; chimeric mouse/human β-amyloid precursor protein
Standard Supply: Level 3

Key Phenotypic Components

These double transgenic mice express a mutant human presenilin 1 (DeltaE9) and a chimeric mouse/human amyloid precursor protein (APPSwe).
The mouse prion protein promoter directs expression of both transgenes.
The DeltaE9 mutation of the human presenilin 1 gene is a deletion of exon 9 and corresponds to a form of early-onset Alzheimer's disease. Human presenilin protein, which in high levels displaces detectable endogenous mouse protein, is immunodetected in whole brain protein homogenates.
Human amyloid precursor protein is also immunodetected in whole brain protein homogenates.
Donating investigator reports transgenic mice develop beta-amyloid deposits in the brains of transgenic animals by 6 to 7 months of age.

Primary Reference
Jankowsky JL, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Borchelt DR. 2001. Co-expression of multiple transgenes in mouse CNS: a comparison of strategies. Biomol Eng 17:157-65. Abstract

For more information, see the data sheet for this strain.

For more information about neurobiology models and other related resources, please visit our Neurobiology Research Models Web page.

APP-PSEN1 double Tg now available

• These double transgenic mice express both a chimeric mouse/human amyloid precursor protein (APPswe) and a mutant human presenilin 1 (DeltaE9). b-amyloid deposits are detected in the brains of transgenic animals by 4 to 6 months of age

Primary Reference
Jankowsky JL, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Borchelt DR. 2001. Co-expression of multiple transgenes in mouse CNS: a comparison of strategies. Biomol Eng 17 :157-65. [Abstract]

For more information, see the data sheet for this strain.

Strain Name: B6.129S7-Per2^tm1Brd
Strain Common Name: mPer2^Brdm1
Stock Number: 003819
Gene Symbol: Per2
Gene Name: period homolog 2 (Drosophila)
Common Names: mper2
Standard Supply: Level 4

Key Phenotypic Components

• When maintained in constant darkness, homozygous mutant mice display a shortened circadian period and a loss of persistent circadian rhythmicity.

Primary Reference
Zheng B, Larkin DW, Albrecht U, Sun ZS, Sage M, Eichele G, Lee CC, Bradley A 1999. The mPer2 gene encodes a functional component of the mammalian circadian clock. Nature 400:169-73. [PubMed: 99334929]

For more information, see the data sheet for this strain.

2. A Mouse Model of Fragile X Syndrome

Strain Name: FVB.129P2-Fmr1^tm1Cgr
Strain Common Name: FraX
Stock Number: 004624
Gene Symbol: Fmr1
Gene Name: fragile X mental retardation syndrome 1 homolog
Standard Supply: Under Development, please register interest

Key Phenotypic Components

• This strain carries a deletion of the gene associated with Fragile X syndrome.
  
• This strain has been backcrossed onto an FVB genetic background and is homozygous for the 129P2/OlaHsd wildtype Pde6b allele, and therefore does not suffer from blindness due to retinal degeneration.

Primary Reference
The Dutch-Belgian Fragile X Consortium. (Bakker CE, Verheij C, Willemsen R, van der Helm R, Oerlemans F, Vermey M, Bygrave A, Hoogeveen AT, Oostra BA, Reyniers E, et al). 1994. Fmr1 knockout mice: a model to study fragile X mental retardation. Cell 78:23-33. [PubMed: 8033209]

For more information, see the data sheet for this strain.

3. A Mouse for Studying Angelman Syndrome

Strain Name: 129-Ube3a^tm1Alb
Stock Number: 004477
Gene Symbol: Ube3a
Gene Name: ubiquitin protein ligase E3A
Common Names: E6-AP ubiquitin protein ligase, Hpve6a; ubiquitin conjugating enzyme E3A
Standard Supply: Under Development, please register interest

Key Phenotypic Components

• Due to imprinting, heterozygous mice with a maternal deficiency display the phenotype while heterozygous mice with a paternal deficiency do not.
  
• The phenotype of mice with maternal deficiency resembles that of human Angelman syndrome with motor dysfunction, inducible seizures, and a context-dependent learning deficit.

Primary Reference
Jiang YH, Armstrong D, Albrecht U, Atkins CM, Noebels JL, Eichele G, Sweatt JD, Beaudet AL 1998. Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation. Neuron 21:799-811. Abstract.

For more information, see the data sheet for this strain.

4. A Mouse for Stress, Anxiety, Anorexia and Fetal Distress Syndrome Studies

Strain Name: B6;129-Crhr1^tm1Klee
Strain Common Names: CRF-R1 -/-; CRFR1 null; CRFR1-Deficient
Stock Number: 004454
Gene Symbol: Crhr1
Gene Name: corticotropin releasing hormone receptor 1
Common Names: CRF-R1alpha; CRFR1; Crhr
Standard Supply: Under Development, please register interest

Key Phenotypic Components

• Mutants have very low plasma corticosterone levels, and no diurnal rise in levels.
  
• Homozygous mice display reduced anxiety response behavior. Hormonal response to stress is diminished in homozygous mice due to impairment of the hypothalamic-pituitary-adrenal axis.
  
• Mutant mice have impaired spatial recognition memory.

Primary Reference
Smith GW, Aubry JM, Dellu F, Contarino A, Bilezikjian LM, Gold LH, Chen R, Marchuk Y, Hauser C, Bentley CA, Sawchenko PE, Koob GF, Vale W, Lee KF 1998. Corticotropin releasing factor receptor 1-deficient mice display decreased anxiety, impaired stress response, and aberrant neuroendocrine development. Neuron 20:1093-102. [PubMed: 98318230]

For more information, see the data sheet for this strain.

Two new mouse lines that allow regulated mutagenesis are Under Development at The Jackson Laboratory. These include floxed alleles of a key GABA receptor subunit and of the Bdnf gene.

1. A Research Tool for Studying the Role of the alpha1 subunit of the GABA Receptor:
Strain Name: B6.129(FVB)-Gabra1^tm1Geh
Stock Number: 004318
Symbol: Gabra1
Gene Name: gamma-aminobutyric acid (GABA-A) receptor, subunit alpha 1
Common Names: Gabra-1
Standard Supply - Under Development: Estimated Available for Sale Date June 16, 2003 (please register interest)

Key Phenotypic Components:

• These mice possess loxP sites on either side of the Gabra1 exon that encodes the second transmembrane domain.
  
• When used in conjunction with Cre recombinase-expressing strains, this line is useful in generating tissue-specific and/or developmentally-regulated knockouts of the alpha1 subunit of the GABA receptor.

Primary Reference:
Vicini S, Ferguson C, Prybylowski K, Kralic J, Morrow AL, Homanics GE. 2001. GABA(A) receptor alpha1 subunit deletion prevents developmental changes of inhibitory synaptic currents in cerebellar neurons. J Neurosci 21 :3009-16. Abstract

For more information, visit the data sheet for this strain at: http://jaxmice.jax.org/micedata.shtml?stock=004318.

2. A Research Tool for Studying the Role of Brain-Derived Neurotrophic Factor (BDNF):

Strain Name: STOCK Bdnf ^tm2Jae
Common Name: Bdnf ^2lox
Stock Number: 004339
Symbol: Bdnf
Gene Name: brain derived neurotrophic factor
Standard Supply - Under Development: please register interest

Key Phenotypic Components:

• These mice possess loxP sites on either side of exon 5 of the Bdnf gene.
• When used in conjunction with Cre recombinase-expressing strains, this line is useful in generating tissue-specific and/or developmentally-regulated knockouts of Bdnf.

Primary Reference:
Rios M, Fan G, Fekete C, Kelly J, Bates B, Kuehn R, Lechan RM, Jaenisch R. 2001. Conditional deletion of brain-derived neurotrophic factor in the postnatal brain leads to obesity and hyperactivity. Mol Endocrinol 15 :1748-57. Abstract

For more information, visit the data sheet for this strain at: http://jaxmice.jax.org/micedata.shtml?stock=004339.

Posted 11 April 2003

Six mouse models for studying Alzheimer's Disease are newly available or Under Development at The Jackson Laboratory. These include a knockout of the mouse amyloid β precurser protein (App) gene and two transgenics expressing a variant of human APP that leads to amyloid deposits. Three of these models have the mouse apolipoprotein (Apoe) gene knocked out and express a human apolipoprotein E allele (APOE2, APOE3, or APOE 4). Information about each of these models is provided below. For full details, refer to the corresponding strain data sheets at our Web site.

Some of these mouse models exhibit the phenotype at a certain age. JAX Services can deliver mice to you at the age you require.

1. A Mouse Model for Studies Related to Alzheimer's Disease

Strain Name: B6.129S7-App^tm1Dbo
Gene Name: amyloid β (A4) precursor protein
Stock Number: 004133
Standard Supply: Level 4

Key Phenotypic Components:

• No App gene product (mRNA or protein) is detected.
• By 14 weeks of age the mice exhibit evidence of reactive gliosis.
• Neurological evaluation reveals significantly reduced forelimb grip strength and decreased locomotor activity.

Primary Reference:
Zheng H, Jiang M, Trumbauer ME, Sirinathsinghji DJ, Hopkins R, Smith DW, Heavens RP, Dawson GR, Boyce S, Conner MW, et al. 1995. β-Amyloid precursor protein-deficient mice show reactive gliosis and decreased locomotor activity. Cell 81 :525-31. Abstract

For more information, see the data sheet for this strain.

2. A Mouse Model for Studying Mechanisms of Amyloid Deposition

Strain Name: C3B6-Tg (APP695)3Dbo
Gene Name: amyloid β (A4) precursor protein (chimeric)
Stock Number: 003375
Standard Supply: Under Development, please register interest

Key Phenotypic Components:

• The transgenic construct contains a cDNA encoding a chimeric amyloid β (A4) precursor protein (APP695) regulated by the mouse prion promoter. The chimeric APP695 molecule was created by replacing sequences encoding the Aβ domain of the murine sequence with the cognate sequences of the human gene (mutations K595N and M596L).
• Mice expressing this transgene develop amyloid deposits in brain tissue by 18-20 months of age.

Primary Reference:
Borchelt DR, Thinakaran G, Eckman CB, Lee MK, Davenport F, Ratovitsky T, Prada CM, Kim G, Seekins S, Yager D, Slunt HH, et al. 1996. Familial Alzheimer's disease-linked presenilin 1 variants elevate Aβ1-42/1-40 ratio in vitro and in vivo. Neuron 17 :1005-13. Abstract

For more information, see the data sheet for this strain.
Register an interest in this strain

3. A Mouse Model for Alzheimer's Research

Strain Name: B6C3-Tg(APP695)85Dbo Tg(PSEN1)85Dbo
Strain Number: 004462
Gene Names: APP; PSEN1
Standard Supply" under development, please register interest

Key Phenotypic Components:

• These double transgenic mice express both a chimeric mouse/human amyloid precursor protein (APPswe) and a mutant human presenilin 1 (DeltaE9)
• β-amyloid deposits are detected in the brains of transgenic animals by 4 to 6 months of age

Primary Reference:
Jankowsky JL, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Borchelt DR. 2001. Co-expression of multiple transgenes in mouse CNS: a comparison of strategies. Biomol Eng 17 :157-65. Abstract

For more information, see the data sheet for this strain.
Register an interest in this strain.

Three Mouse Models for Studying the Role of Human Apolipoprotein E in Alzheimer's Disease

4. Strain Name: B6.Cg-Tg(GFAP-APOE2)14Hol Apoe^tm1Unc
Stock Number: 004632

5. Strain Name: B6.Cg-Tg(GFAP-APOE3)37Hol Apoe^tm1Unc
Stock Number: 004633

6. Strain Name: B6.Cg-Tg(GFAP-APOE4)1Hol Apoe^tm1Unc
Stock Number: 004631

Gene Name: apolipoprotein E (Human) Standard Supply: Under Development, please register interest

Key Phenotypic Components:

• These three transgenic mice strains express the human apolipoprotein E2, E3, or E4 isoform under the direction of the human glial fibrillary acidic protein (GFAP) promoter, and do not express endogenous mouse apolipoprotein E (APOE).
• Cultured astrocytes from transgenic mice secrete the APOE isoform in lipoproteins that are similar in size to high-density (HDL) plasma lipoproteins, and which enhance hippocampal neurite outgrowth.
• Detergent-soluble APOE protein level in forebrain of hemizygous mice is similar to that in adult human cortex tissue.

Primary Reference:
Sun Y, Wu S, Bu G, Onifade MK, Patel SN, LaDu MJ, Fagan AM, Holtzman DM. 1998. Glial fibrillary acidic protein-apolipoprotein E (apoE) transgenic mice: astrocyte-specific expression and differing biological effects of astrocyte-secreted apoE3 and apoE4 lipoproteins. J Neurosci 18 :3261-72. Abstract

For more information, see the data sheet for these strains at:
APOE2
APOE3
APOE4

To register an interest in these strains, visit:
APOE2
APOE3
APOE4

The Jackson Laboratory has appointed a special research administrator for Alzheimer's disease (AD) to enhance the distribution of mouse models important to AD research. Dr. Sasner will serve as a resource for scientists seeking information about existing models, and also oversee the importation, rederivation, and distribution of new AD models. For information on AD-relevant strains, visit the Neurobiology Research Models page. Scientists with questions or information about potential new strains for distribution can reach Dr. Sasner by e-mail (Dr. Michael Sasner).

Jackson is currently developing a distribution colony of David Borchelt's co-injected APPswe/PSEN1(deltaE9) strain. Those interested in this model are urged to place an advance order so that an appropriate number of animals can be bred for the distribution colony. Contact Customer Service (800-422-MICE or orderquest@jax.org) or register online
Strain Name: B6C3-Tg(APP695)85Dbo Tg(PSEN1)85Dbo Stock Number: 004462

The harlequin mouse (B6CBACa Aw-J/A-Pdcd8Hq Stock Number: 000501), which has an interesting phenotype including increased oxidative stress, re-expression of cell cycle genes and neuronal death (see related news story) is now available for purchase.