Strain Name:

B6.Cg-Fbn1Tsk/J

Stock Number:

014632

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Availability:

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Fbn1Tsk homozygotes are embryonic lethal; heterozygotes exhibit excessive growth and hyperplasia of connective tissue, cartilage, tendon sheaths and bone. This strain is a model for Marfan Syndrome, Hereditary Pulmonary Emphysema, Stiff Skin Syndrome and Familial Progressive Scleroderma.

Description

Strain Information

Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Mating SystemInbred x Heterozygote         (Female x Male)   11-DEC-12
Specieslaboratory mouse
GenerationN5 (29-OCT-13)
Generation Definitions
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Description
The Fbn1Tsk allele contains a 30 to 40 kbp genomic tandem duplication resulting in a larger than normal in-frame transcript. Homozygotes are embryonic lethal, failing to survive past somite formation (7-8 days of gestation). Heterozygotes are viable and fertile, exhibiting excessive growth and hyperplasia of connective tissue, cartilage, tendon sheaths and bone. Skin tightness, due to hyperplasic thickening of subcutaneous loose connective tissue and abnormal organization and distribution of skin microfibrillar arrays, develops by the first week after birth. Although the size of the skeleton is increased, body weight remains normal. Mutant mice exhibit polyuria during the light cycle. Collagens and glycosaminoglycans accumulate in the skin, heart, lungs and bladder. Hypertrophy is also observed in the enlarged heart (aortic adventitia). Mutant mice have enlarged thoracic size and lungs with abnormal alveolar walls, irregular shaped alveoli, and increased lung capacity. By 1 month of age, heterozygotes develop emphysema with progressive destruction of alveolar walls and loss of elasticity. Inflammatory macrophages and neutrophils infiltrate the lower respiratory tract. The abnormal immune system characteristics exhibited by this strain also include: alveolitis, increased number of mast cells in the skin, presence of autoantibodies specific for scleroderma target antigens and anti-nuclear antibodies.

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 description if necessary as published results become available.

Development
The spontaneous mutation Fbn1Tsk (tight skin) arose in 1977 at The Jackson Laboratory from the B10.D2(58N) congenic resistant strain.
This strain was generated by breeding B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/J (Stock No. 000305) to C57BL6/J (Stock No. 000664) to remove the Bloc1s6pa allele.

A 32 SNP (single nucleotide polymorphism) panel analysis, with 27 markers covering all 19 chromosomes and the X chromosome, as well as 5 markers that distinguish between the C57BL/6J and C57BL/6N substrains, was performed on the rederived living colony at The Jackson Laboratory Repository. While the 27 markers throughout the genome suggested a C57BL/6 genetic background, 4 of 5 markers that determine C57BL/6J from C57BL/6N were found to be segregating. These data suggest the mice sent to The Jackson Laboratory Repository were on a mixed C57BL/6J ; C57BL/6N genetic background.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Fbn1Tsk allele
000305   B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/J
View Strains carrying   Fbn1Tsk     (1 strain)

Strains carrying other alleles of Fbn1
012885   B6.129-Fbn1tm1Hcd/J
005704   B6.129-Fbn1tm2Rmz/J
View Strains carrying other alleles of Fbn1     (2 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Marfan Syndrome; MFS
Models with phenotypic similarity to human diseases where etiology is unknown or involving genes where ortholog is unknown.
Emphysema, Hereditary Pulmonary
Scleroderma, Familial Progressive
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Pituitary Adenoma, Growth Hormone-Secreting
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Acromicric Dysplasia; ACMICD   (FBN1)
Ectopia Lentis 1, Isolated, Autosomal Dominant; ECTOL1   (FBN1)
Geleophysic Dysplasia 2; GPHYSD2   (FBN1)
MASS Syndrome   (FBN1)
Stiff Skin Syndrome; SSKS   (FBN1)
Weill-Marchesani Syndrome 2; WMS2   (FBN1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Fbn1Tsk/Fbn1+

        B6.Cg-Fbn1Tsk
  • respiratory system phenotype
  • abnormal lung morphology   (MGI Ref ID J:30961)
    • abnormal bronchiole morphology
      • dilation of bronchioles   (MGI Ref ID J:30961)
    • abnormal pulmonary alveolus morphology   (MGI Ref ID J:3934)
      • thinning and destruction of alveolar walls   (MGI Ref ID J:30961)
      • numerous broken alveolar septa and bullous lesions   (MGI Ref ID J:30961)
      • alveoli are irregular in size, with most appearing enlarged   (MGI Ref ID J:30961)
      • at 3 weeks of age, alveoli are flattened but not enlarged, however by 4-6 weeks af age, alveoli are enlarged   (MGI Ref ID J:15018)
      • exhibit formation of bullae and subpleural cysts and fragmented elastin in alveolar walls   (MGI Ref ID J:1326)
      • enlargement of air spaces with numerous subpleural cysts and scattered bullae   (MGI Ref ID J:6566)
      • abnormal pulmonary alveolus wall morphology
        • thinning and destruction of alveolar walls   (MGI Ref ID J:30961)
      • overexpanded pulmonary alveoli
        • distension of many alveoli   (MGI Ref ID J:30961)
        • develop enlarged alveoli between 4 and 6 weeks of age   (MGI Ref ID J:15018)
        • enlarged alveoli are 3 to 4 times larger than normal and show a histologic picture of emphysema   (MGI Ref ID J:68448)
    • abnormal pulmonary interalveolar septum morphology
      • progressive destruction of alveolar septa and increase in collagen deposition in the septa that may result from repair of the lung destruction   (MGI Ref ID J:3934)
      • abnormal alveolar pore morphology
        • increase in the number and size of the pores of Kohn   (MGI Ref ID J:6566)
        • alveolar pores in septa are variable in size and increased in number   (MGI Ref ID J:30961)
      • thick pulmonary interalveolar septum
        • diffuse thickening of the septa not affected by emphysematous changes, resulting from a progressive increase in collagen   (MGI Ref ID J:3934)
    • dilated pulmonary alveolar ducts
      • dilation of alveolar ducts   (MGI Ref ID J:30961)
      • enlarged alveolar ducts are first seen at 3 weeks of age   (MGI Ref ID J:15018)
    • emphysema   (MGI Ref ID J:20724)
      • emphysematous lesions are seen at 4 weeks of age   (MGI Ref ID J:15018)
  • increased lung compliance   (MGI Ref ID J:6566)
    • increase in lung compliance   (MGI Ref ID J:30961)
  • increased total lung capacity   (MGI Ref ID J:6566)
    • increase in total lung capacity   (MGI Ref ID J:30961)
  • lung inflammation
    • increased numbers of alveolar macrophages and neutrophils in the interstitium   (MGI Ref ID J:30961)
    • presence of focal collections of mononuclear phagocytes, lymphocytes, and neutrophils in the interstitium   (MGI Ref ID J:6566)
    • alveolitis
      • exhibit alveolitis, with increased numbers of alveolar macrophages and neutrophils in the alveolar lumens   (MGI Ref ID J:30961)
      • macrophage-neutrophil alveolitis is seen at 3 weeks of age, at a time when emphysematous lesions are not yet present   (MGI Ref ID J:15018)
      • presence of focal collections of mononuclear phagocytes, lymphocytes, and neutrophils within alveolar air spaces   (MGI Ref ID J:6566)
  • immune system phenotype
  • abnormal uterine NK cell morphology
    • distribution of granulated metrial gland cells, a uterine natural killer-like cell subset, is abnormal during pregnancy, with cells seen in the antimesometral and lateral decidual regions at E8.5 and in regions between implantation sites until E10.5, however their differentiation progresses normally   (MGI Ref ID J:1377)
  • increased autoantibody level
    • develop anti-nucleolar antibodies and produce significantly higher titers of autoantibodies specific for scleroderma target antigens (topo I, RNA pol I, and Fc gamma R)   (MGI Ref ID J:21987)
    • develop autoantibodies specific for scleroderma target antigens, with a bias toward the use of VHJ558 genes and JH2 and JK2 segments   (MGI Ref ID J:14166)
  • increased mast cell number
    • increase in number and enhanced degree of degranulation of mast cells in the skin   (MGI Ref ID J:8047)
  • increased susceptibility to type IV hypersensitivity reaction
    • develop cell-mediated immunity to elastase-soluble murine lung peptides with age while delayed-type hypersensitivity responses to type I or type IV collagen are not detected   (MGI Ref ID J:7185)
  • lung inflammation
    • increased numbers of alveolar macrophages and neutrophils in the interstitium   (MGI Ref ID J:30961)
    • presence of focal collections of mononuclear phagocytes, lymphocytes, and neutrophils in the interstitium   (MGI Ref ID J:6566)
    • alveolitis
      • exhibit alveolitis, with increased numbers of alveolar macrophages and neutrophils in the alveolar lumens   (MGI Ref ID J:30961)
      • macrophage-neutrophil alveolitis is seen at 3 weeks of age, at a time when emphysematous lesions are not yet present   (MGI Ref ID J:15018)
      • presence of focal collections of mononuclear phagocytes, lymphocytes, and neutrophils within alveolar air spaces   (MGI Ref ID J:6566)
  • hematopoietic system phenotype
  • abnormal uterine NK cell morphology
    • distribution of granulated metrial gland cells, a uterine natural killer-like cell subset, is abnormal during pregnancy, with cells seen in the antimesometral and lateral decidual regions at E8.5 and in regions between implantation sites until E10.5, however their differentiation progresses normally   (MGI Ref ID J:1377)
  • increased mast cell number
    • increase in number and enhanced degree of degranulation of mast cells in the skin   (MGI Ref ID J:8047)
  • cardiovascular system phenotype
  • abnormal aorta tunica adventitia morphology
    • aorta exhibits hyperplasia of loose connective tissue in the adventitia   (MGI Ref ID J:1326)
    • collagen fibers in the aorta are increased and microfibrils surrounding elastin in the adventitia of the aorta are not clearly apparent   (MGI Ref ID J:1326)
  • abnormal heart morphology
    • collagen deposition is increased in the heart   (MGI Ref ID J:24523)
    • type I collagen is increased in the myocardium, perhaps due to reduced activity of negative regulatory sequence   (MGI Ref ID J:24523)
    • 2.5-fold increase in type VI collagen content in myocardium   (MGI Ref ID J:17160)
    • abnormal heart right ventricle morphology
      • increase in collagen content in the right ventricle at 3 months of age; however, by 16 months of age, collagen content returns to normal levels but there is a shift in collagen type due to an increase in type I collagen, and by 24 months of age, again see an increase in collagen content   (MGI Ref ID J:20724)
      • heart right ventricle hypertrophy
        • starts to develop at around 8 months of age   (MGI Ref ID J:20724)
    • cardiac fibrosis
      • exhibit myocardial fibrosis   (MGI Ref ID J:24523)
      • thyroid hormone treatment decreases collagen synthesis and stimulates regression of cardiac fibrosis   (MGI Ref ID J:1562)
  • renal/urinary system phenotype
  • abnormal urinary bladder morphology
    • 70% increase in collagen content and concentration in the bladder at 5-6 months of age   (MGI Ref ID J:3616)
  • abnormal urinary bladder physiology
    • functional bladder capacity appears to be greater   (MGI Ref ID J:3616)
  • polyuria
    • urinate larger volumes more frequently during the light cycle   (MGI Ref ID J:3616)
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • no aberrant bleeding time after tail vein nick   (MGI Ref ID J:29151)
  • reproductive system phenotype
  • abnormal uterine NK cell morphology
    • distribution of granulated metrial gland cells, a uterine natural killer-like cell subset, is abnormal during pregnancy, with cells seen in the antimesometral and lateral decidual regions at E8.5 and in regions between implantation sites until E10.5, however their differentiation progresses normally   (MGI Ref ID J:1377)
  • integument phenotype
  • abnormal hypodermis morphology
    • increase in width of the subcutaneous fibrous layer with increasing age   (MGI Ref ID J:8047)
  • abnormal skin morphology
    • significantly greater skin biopsy weights, however percent of water-fat is similar to wild-type   (MGI Ref ID J:7057)
    • hexosamine, uronic acid, and total glycosaminoglycan content is increased in skin   (MGI Ref ID J:7057)
    • abnormal cutaneous collagen fibril morphology
      • electron micrographs of skin show a predominance of abnormally small diameter collagen fibers and the skin shows abundant irregular and wavy collagen bundles   (MGI Ref ID J:68448)
    • abnormal cutaneous microfibril morphology
      • transmission electron microscopy of the upper dermis shows more prominent microfibrillar clusters, which often appear blurred and lacking a discernible striated pattern, but an absence of well-packed elastic fibrils   (MGI Ref ID J:46405)
      • although there is a morphologically normal population of skin microfibrils, approximately 45% of the skin microfibril population has abnromal periodic arrays of beads with indistinct filamentous interbeads and extended periodicity of 112 (+/-11) nm relative to 55 (+/-4) nm in normal microfibrils   (MGI Ref ID J:46405)
      • the abnormal skin microfibrils have an altered response to calcium chelation by EDTA, with diminished shortening of the periodicity of microfibrils and less prominant appearance of beading compared with control microfibrils, and the large microfibril aggregates fail to dissociate   (MGI Ref ID J:46405)
    • skin fibrosis   (MGI Ref ID J:68448)
      • develop skin fibrosis which results originally from collagen I and III overexpression and later collagen VI overexpression   (MGI Ref ID J:26096)
      • unlike human scleroderma, fibrosis does not manifest Tgfb1 mRNA in areas of abnormal collagen deposition   (MGI Ref ID J:26096)
    • thick skin
      • skin is 228 um thick on average instead of the wildtype 132 um   (MGI Ref ID J:68448)
  • abnormal skin physiology
    • cultured skin fibroblasts synthesize almost 5 times more Type I and Type III procollagen mRNA indicating production of excessive amounts of collagen   (MGI Ref ID J:8140)
    • fibroblasts synthesize increased collagen both constitutively and in response to IL4 or TGFB, although IL13 does not increase fibroblast collagen synthesis   (MGI Ref ID J:68448)
  • embryogenesis phenotype
  • abnormal uterine NK cell morphology
    • distribution of granulated metrial gland cells, a uterine natural killer-like cell subset, is abnormal during pregnancy, with cells seen in the antimesometral and lateral decidual regions at E8.5 and in regions between implantation sites until E10.5, however their differentiation progresses normally   (MGI Ref ID J:1377)

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Fbn1Tsk/Fbn1+

        B10.D2/(58N)Sn
  • skeleton phenotype
  • abnormal skeleton morphology
    • excessive growth of connective tissue and skeleton   (MGI Ref ID J:5629)
    • increase in skeletal size, however body weight is not increased   (MGI Ref ID J:5629)
    • abnormal cartilage morphology
      • increase in growth of cartilage   (MGI Ref ID J:5629)
      • longer length of the ear cartilage   (MGI Ref ID J:5629)
      • abnormal costal cartilage morphology
        • costal cartilages are elongated and more bowed than normal   (MGI Ref ID J:5629)
      • abnormal tracheal cartilage morphology
        • longer length of the fourth tracheal ring   (MGI Ref ID J:5629)
    • abnormal pelvic girdle bone morphology
      • pelvic bone is about 10% larger   (MGI Ref ID J:5629)
    • abnormal tendon morphology
      • small tendons with hyperplasia of tendon sheaths or with accumulation of fluid within the sheath   (MGI Ref ID J:5629)
      • in older mice, some tendons show degenerative changes with increased cellularity and decreased amount of collagen   (MGI Ref ID J:5629)
    • enlarged cranium
      • longer skull   (MGI Ref ID J:5629)
    • enlarged lumbar vertebrae
      • enlarged in both length and width   (MGI Ref ID J:5629)
    • increased length of long bones
      • long bones and girdles are about 5% larger   (MGI Ref ID J:5629)
    • long mandible
      • longer and wider mandible   (MGI Ref ID J:5629)
    • long ribs
      • longer ribs   (MGI Ref ID J:5629)
  • cardiovascular system phenotype
  • dilated heart atrium   (MGI Ref ID J:5629)
  • dilated heart right ventricle
    • enlarged but not as much as the auricles   (MGI Ref ID J:5629)
  • enlarged heart   (MGI Ref ID J:5629)
    • cardiac hypertrophy   (MGI Ref ID J:32931)
  • respiratory system phenotype
  • abnormal lung morphology
    • lungs become abnormally distended in enlarged thorax   (MGI Ref ID J:5629)
    • emphysema
      • vesicular emphysema   (MGI Ref ID J:5629)
  • abnormal tracheal cartilage morphology
    • longer length of the fourth tracheal ring   (MGI Ref ID J:5629)
  • muscle phenotype
  • abnormal tendon morphology
    • small tendons with hyperplasia of tendon sheaths or with accumulation of fluid within the sheath   (MGI Ref ID J:5629)
    • in older mice, some tendons show degenerative changes with increased cellularity and decreased amount of collagen   (MGI Ref ID J:5629)
  • behavior/neurological phenotype
  • hunched posture
    • develop a pronounced hump in the shoulder region and hunched posture with age   (MGI Ref ID J:5629)
  • craniofacial phenotype
  • enlarged cranium
    • longer skull   (MGI Ref ID J:5629)
  • long mandible
    • longer and wider mandible   (MGI Ref ID J:5629)
  • growth/size/body phenotype
  • enlarged thoracic cavity
    • enlarged thorax causing distension of the thoracic viscera   (MGI Ref ID J:5629)
  • integument phenotype
  • abnormal hypodermis morphology
    • hyperplasia of the subcutaneous loose connective tissue   (MGI Ref ID J:5629)
    • in loose connective tissue, exhibit large accumulations of microfibrils in the intercellular space   (MGI Ref ID J:5629)
    • hypodermis is more lamellar   (MGI Ref ID J:6273)
    • fascicles of unusually thin collagen fibrils are found in scattered areas of the hypodermis   (MGI Ref ID J:6273)
    • thick hypodermis
      • hypodermis is substantially thicker   (MGI Ref ID J:6273)
  • abnormal skin morphology   (MGI Ref ID J:6273)
    • abnormal dermal layer morphology
      • dermis fibroblasts often contain greatly distended rough endoplasmic reticulum cisternae   (MGI Ref ID J:6273)
      • collagen fibrils within the fascicles of the dermis are less ordered and the fascicles are thinner and more closely packed and appear to bend and twist more along their course   (MGI Ref ID J:6273)
      • abnormal dermis papillary layer morphology
        • fibrous organization of collagen fibrils is not distinctly visible in the hyalinized areas of the superficial dermis   (MGI Ref ID J:6273)
      • thick dermal layer
        • reticular dermis of skin is consistently thicker and often more cellular than that of wild-type   (MGI Ref ID J:6273)
    • abnormal skin condition   (MGI Ref ID J:27521)
    • tight skin
      • caused by hyperplasia of subcutaneous loose connective tissue   (MGI Ref ID J:27521)
      • skin is firmly bound to subcutaneous and deep muscular tissue   (MGI Ref ID J:32931)
      • skin lacks normal pliability and elasticity   (MGI Ref ID J:32931)
      • skin tightness is not seen at birth but develops during the first postnatal week   (MGI Ref ID J:5629)

Fbn1Tsk/Fbn1+

        B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/JKcc
  • cardiovascular system phenotype
  • abnormal aorta elastin content
    • in 3 week old mice fragmentation of elastic lamellae is seen in van-Gieson stained histological sections of the ascending aorta   (MGI Ref ID J:63844)
  • integument phenotype
  • abnormal dermal layer morphology   (MGI Ref ID J:63844)
    • abnormal dermis reticular layer morphology
      • Trichrome-stained sections of adult skin show loose connective tissue contains excessive collagen fibers tightly bound to the muscle layer   (MGI Ref ID J:63844)
      • abnormal dermis reticular layer collagen network
        • Trichrome-stained sections of adult skin show loose connective tissue contains excessive collagen fibers tightly bound to the muscle layer   (MGI Ref ID J:63844)

Fbn1Tsk/Fbn1Tsk

        B10.D2/(58N)Sn
  • mortality/aging
  • complete embryonic lethality between implantation and somite formation
    • die at 7-8 days of gestation   (MGI Ref ID J:5629)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Cardiovascular Research
Heart Abnormalities

Cell Biology Research
Genes Regulating Growth and Proliferation

Dermatology Research
Skin and Hair Texture Defects

Developmental Biology Research
Embryonic Lethality (Homozygous)
Growth Defects
Skeletal Defects

Immunology, Inflammation and Autoimmunity Research
Autoimmunity
Inflammation

Internal/Organ Research
Lung Defects
      emphysema
Skeleton
      Bone

Research Tools
Cardiovascular Research
Immunology, Inflammation and Autoimmunity Research

Fbn1Tsk related

Dermatology Research
Skin and Hair Texture Defects

Developmental Biology Research
Defects in Extracellular Matrix Molecules

Internal/Organ Research
Lung Defects
      emphysema

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Fbn1Tsk
Allele Name tight skin
Allele Type Spontaneous
Common Name(s) TSK;
Strain of OriginB10.D2/(58N)Sn
Gene Symbol and Name Fbn1, fibrillin 1
Chromosome 2
Gene Common Name(s) ACMICD; AI536462; ECTOL1; FBN; Fib-1; GPHYSD2; MASS; MFS1; OCTD; SGS; SSKS; Tsk; WMS; WMS2; expressed sequence AI536462; tight skin;
General Note Genbank ID for this allele: AF007248
Molecular Note This allele harbors a 30 to 40kb genomic tandem duplication within the Fbn1 gene that results in a larger than normal in-frame transcript produced at normal levels. [MGI Ref ID J:32931] [MGI Ref ID J:45733] [MGI Ref ID J:55886]

Genotyping

Genotyping Information

Genotyping Protocols

Fbn1Tsk, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Akita M; Lee SH; Kaneko K. 1992. Electron microscopic observations of elastic fibres in the lung and aorta of tight-skin and beta-aminopropionitrile-fed mice. Histol Histopathol 7(1):39-45. [PubMed: 1576433]  [MGI Ref ID J:1326]

Green MC; Sweet HO. 1973. Tight skin (Tsk) Mouse News Lett 48:34.  [MGI Ref ID J:27521]

Green MC; Sweet HO; Bunker LE. 1976. Tight-skin, a new mutation of the mouse causing excessive growth of connective tissue and skeleton. Am J Pathol 82(3):493-512. [PubMed: 176891]  [MGI Ref ID J:5629]

Rossi GA; Hunninghake GW; Gadek JE; Szapiel SV; Kawanami O; Ferrans VJ; Crystal RG. 1984. Hereditary emphysema in the tight-skin mouse. Evaluation of pathogenesis. Am Rev Respir Dis 129(5):850-5. [PubMed: 6562869]  [MGI Ref ID J:15018]

Siracusa LD; McGrath R; Ma Q; Moskow JJ; Manne J; Christner PJ; Buchberg AM; Jimenez SA. 1996. A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation. Genome Res 6(4):300-13. [PubMed: 8723723]  [MGI Ref ID J:32931]

Szapiel SV; Fulmer JD; Hunninghake GW; Elson NA; Kawanami O; Ferrans VJ; Crystal RG. 1981. Hereditary emphysema in the tight-skin (Tsk/+) mouse. Am Rev Respir Dis 123(6):680-5. [PubMed: 7271067]  [MGI Ref ID J:6566]

Additional References

Fbn1Tsk related

Akiyama K; Chen C; Wang D; Xu X; Qu C; Yamaza T; Cai T; Chen W; Sun L; Shi S. 2012. Mesenchymal-stem-cell-induced immunoregulation involves FAS-ligand-/FAS-mediated T cell apoptosis. Cell Stem Cell 10(5):544-55. [PubMed: 22542159]  [MGI Ref ID J:185809]

Alexander JJ; Jacob A; Cunningham P; Hensley L; Quigg RJ. 2008. TNF is a key mediator of septic encephalopathy acting through its receptor, TNF receptor-1. Neurochem Int 52(3):447-56. [PubMed: 17884256]  [MGI Ref ID J:140283]

Asano N; Fujimoto M; Yazawa N; Shirasawa S; Hasegawa M; Okochi H; Tamaki K; Tedder TF; Sato S. 2004. B Lymphocyte signaling established by the CD19/CD22 loop regulates autoimmunity in the tight-skin mouse. Am J Pathol 165(2):641-50. [PubMed: 15277237]  [MGI Ref ID J:91521]

Bashey RI; Philips N; Insinga F; Jimenez SA. 1993. Increased collagen synthesis and increased content of type VI collagen in myocardium of tight skin mice. Cardiovasc Res 27(6):1061-5. [PubMed: 8221765]  [MGI Ref ID J:17160]

Baxter RM; Crowell TP; McCrann ME; Frew EM; Gardner H. 2005. Analysis of the tight skin (Tsk1/+) mouse as a model for testing antifibrotic agents. Lab Invest 85(10):1199-209. [PubMed: 16127425]  [MGI Ref ID J:103677]

Bayle J; Fitch J; Jacobsen K; Kumar R; Lafyatis R; Lemaire R. 2008. Increased expression of Wnt2 and SFRP4 in Tsk mouse skin: role of Wnt signaling in altered dermal fibrillin deposition and systemic sclerosis. J Invest Dermatol 128(4):871-81. [PubMed: 17943183]  [MGI Ref ID J:135507]

Bocchieri MH; Christner PJ; Henriksen PD; Jimenez SA. 1993. Immunological characterization of (tight skin/NZB)F1 hybrid mice with connective tissue and autoimmune features resembling human systemic sclerosis. J Autoimmun 6(3):337-51. [PubMed: 8397715]  [MGI Ref ID J:13260]

Bona CA; Murai C; Casares S; Kasturi K; Nishimura H; Honjo T; Matsuda F. 1997. Structure of the mutant fibrillin-1 gene in the tight skin (TSK) mouse. DNA Res 4(4):267-71. [PubMed: 9405934]  [MGI Ref ID J:45733]

Calvi C; Podowski M; Lopez-Mercado A; Metzger S; Misono K; Malinina A; Dikeman D; Poonyagariyon H; Ynalvez L; Derakhshandeh R; Le A; Merchant M; Schwall R; Neptune ER. 2013. Hepatocyte growth factor, a determinant of airspace homeostasis in the murine lung. PLoS Genet 9(2):e1003228. [PubMed: 23459311]  [MGI Ref ID J:195194]

DeLustro FA; Mackel AM; LeRoy EC. 1983. Delayed-type hypersensitivity to elastase-soluble lung peptides in the tight-skin (Tsk) mouse. Cell Immunol 81(1):175-9. [PubMed: 6604585]  [MGI Ref ID J:7185]

Dimitriu-Bona A; Fillit H. 1993. Studies of the cellular immune response to heparan sulfate proteoglycan in the tight skin mouse. Cell Immunol 150(2):321-32. [PubMed: 8370075]  [MGI Ref ID J:14526]

Dodig TD; Mack KT; Cassarino DF; Clark SH. 2001. Development of the tight-skin phenotype in immune-deficient mice. Arthritis Rheum 44(3):723-7. [PubMed: 11263788]  [MGI Ref ID J:112962]

Everett ET; Pablos JL; Harley RA; LeRoy EC; Norris JS. 1995. The role of mast cells in the development of skin fibrosis in tight-skin mutant mice. Comp Biochem Physiol A Physiol 110(2):159-65. [PubMed: 7704626]  [MGI Ref ID J:24076]

Frondoza C; Jones L; Rose NR; Hatakeyama A; Phelps R; Bona C. 1996. Silicone does not potentiate development of the scleroderma-like syndrome in tight skin (TSK/+) mice. J Autoimmun 9(4):473-83. [PubMed: 8864822]  [MGI Ref ID J:35588]

Gardi C; Cavarra E; Calzoni P; Marcolongo P; de Santi M; Martorana PA; Lungarella G. 1994. Neutrophil lysosomal dysfunctions in mutant C57 Bl/6J mice: interstrain variations in content of lysosomal elastase, cathepsin G and their inhibitors. Biochem J 299(Pt 1):237-45. [PubMed: 8166647]  [MGI Ref ID J:17583]

Gardi C; Martorana PA; Calzoni P; Cavarra E; Marcolongo P; de Santi MM; van Even P; Lungarella G. 1994. Cardiac collagen changes during the development of right ventricular hypertrophy in tight-skin mice with emphysema. Exp Mol Pathol 60(2):100-7. [PubMed: 8070538]  [MGI Ref ID J:20724]

Gardi C; Martorana PA; Calzoni P; van Even P; de Santi MM; Cavarra E; Lungarella G. 1992. Lung collagen synthesis and deposition in tight-skin mice with genetic emphysema. Exp Mol Pathol 56(2):163-72. [PubMed: 1587342]  [MGI Ref ID J:3934]

Gardi C; Martorana PA; van Even P; de Santi MM; Lungarella G. 1990. Serum antielastase deficiency in tight-skin mice with genetic emphysema. Exp Mol Pathol 52(1):46-53. [PubMed: 2307213]  [MGI Ref ID J:150253]

Gayraud B; Keene DR; Sakai LY; Ramirez F. 2000. New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse. J Cell Biol 150(3):667-80. [PubMed: 10931876]  [MGI Ref ID J:63844]

Goldstein C; Liaw P; Jimenez SA; Buchberg AM; Siracusa LD. 1994. Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome. Mamm Genome 5(11):696-700. [PubMed: 7873879]  [MGI Ref ID J:21512]

Granchelli JA; Pollina C; Hudecki MS. 1995. Duchenne-like myopathy in double-mutant mdx mice expressing exaggerated mast cell activity. J Neurol Sci 131(1):1-7. [PubMed: 7561939]  [MGI Ref ID J:26738]

Griesenbach U; Smith SN; Farley R; Singh C; Alton EW. 2008. Validation of nasal potential difference measurements in gut-corrected CF knockout mice. Am J Respir Cell Mol Biol 39(4):490-6. [PubMed: 18458238]  [MGI Ref ID J:154280]

Hasegawa M; Hamaguchi Y; Yanaba K; Bouaziz JD; Uchida J; Fujimoto M; Matsushita T; Matsushita Y; Horikawa M; Komura K; Takehara K; Sato S; Tedder TF. 2006. B-lymphocyte depletion reduces skin fibrosis and autoimmunity in the tight-skin mouse model for systemic sclerosis. Am J Pathol 169(3):954-66. [PubMed: 16936269]  [MGI Ref ID J:112355]

Honda N; Jinnin M; Kira-Etoh T; Makino K; Kajihara I; Makino T; Fukushima S; Inoue Y; Okamoto Y; Hasegawa M; Fujimoto M; Ihn H. 2013. miR-150 down-regulation contributes to the constitutive type I collagen overexpression in scleroderma dermal fibroblasts via the induction of integrin beta3. Am J Pathol 182(1):206-16. [PubMed: 23159943]  [MGI Ref ID J:192341]

Ito S; Bartolak-Suki E; Shipley JM; Parameswaran H; Majumdar A; Suki B. 2006. Early emphysema in the tight skin and pallid mice: roles of microfibril-associated glycoproteins, collagen, and mechanical forces. Am J Respir Cell Mol Biol 34(6):688-94. [PubMed: 16439805]  [MGI Ref ID J:122617]

Jimenez SA; Williams CJ; Myers JC; Bashey RI. 1986. Increased collagen biosynthesis and increased expression of type I and type III procollagen genes in tight skin (TSK) mouse fibroblasts. J Biol Chem 261(2):657-62. [PubMed: 2416757]  [MGI Ref ID J:8140]

Kakizoe E; Shiota N; Tanabe Y; Shimoura K; Kobayashi Y; Okunishi H. 2001. Isoform-selective upregulation of mast cell chymase in the development of skin fibrosis in scleroderma model mice. J Invest Dermatol 116(1):118-23. [PubMed: 11168806]  [MGI Ref ID J:68449]

Kasturi KN; Daian C; Saitoh Y; Muryoi T; Bona CA. 1993. Tight-skin mouse autoantibody repertoire: analysis of VH and VK gene usage. Mol Immunol 30(11):969-78. [PubMed: 7688852]  [MGI Ref ID J:14166]

Kasturi KN; Hatakeyama A; Murai C; Gordon R; Phelps RG; Bona CA. 1997. B-cell deficiency does not abrogate development of cutaneous hyperplasia in mice inheriting the defective fibrillin-1 gene J Autoimmun 10(6):505-17. [PubMed: 9451590]  [MGI Ref ID J:45060]

Kasturi KN; Shibata S; Muryoi T; Bona CA. 1994. Tight-skin mouse an experimental model for scleroderma. Int Rev Immunol 11(3):253-71. [PubMed: 7523551]  [MGI Ref ID J:21987]

Kasturi KN; Yio XY; Bona CA. 1994. Molecular characterization of J558 genes encoding tight-skin mouse autoantibodies: identical heavy-chain variable genes code for antibodies with different specificities. Proc Natl Acad Sci U S A 91(17):8067-71. [PubMed: 8058758]  [MGI Ref ID J:19901]

Keil M; Lungarella G; Cavarra E; van Even P; Martorana PA. 1996. A scanning electron microscopic investigation of genetic emphysema in tight-skin, pallid, and beige mice, three different C57 BL/6J mutants. Lab Invest 74(2):353-62. [PubMed: 8780155]  [MGI Ref ID J:31735]

Kielty CM; Raghunath M; Siracusa LD; Sherratt MJ; Peters R; Shuttleworth CA ; Jimenez SA. 1998. The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils. J Cell Biol 140(5):1159-66. [PubMed: 9490728]  [MGI Ref ID J:46405]

Kiso Y; McBey BA; Croy BA. 1992. Granulated metrial gland cells in the pregnant uterus of mice expressing the collagen mutation tight-skin (Tsk/+). Cell Tissue Res 268(2):393-6. [PubMed: 1617705]  [MGI Ref ID J:1377]

Kodera T; McGaha TL; Phelps R; Paul WE; Bona CA. 2002. Disrupting the IL-4 gene rescues mice homozygous for the tight-skin mutation from embryonic death and diminishes TGF-beta production by fibroblasts. Proc Natl Acad Sci U S A 99(6):3800-5. [PubMed: 11891315]  [MGI Ref ID J:75471]

Lemaire R; Farina G; Bayle J; Dimarzio M; Pendergrass SA; Milano A; Whitfield ML; Lafyatis R. 2010. Antagonistic effect of the matricellular signaling protein CCN3 on TGF-beta- and Wnt-mediated fibrillinogenesis in systemic sclerosis and Marfan syndrome. J Invest Dermatol 130(6):1514-23. [PubMed: 20182440]  [MGI Ref ID J:160094]

Lemaire R; Korn JH; Schiemann WP; Lafyatis R. 2004. Fibulin-2 and fibulin-5 alterations in tsk mice associated with disorganized hypodermal elastic fibers and skin tethering. J Invest Dermatol 123(6):1063-9. [PubMed: 15610515]  [MGI Ref ID J:94185]

Levi-Schaffer F; Nagler A; Slavin S; Knopov V; Pines M. 1996. Inhibition of collagen synthesis and changes in skin morphology in murine graft-versus-host disease and tight skin mice: effect of halofuginone. J Invest Dermatol 106(1):84-8. [PubMed: 8592087]  [MGI Ref ID J:31146]

Longhurst PA; Eika B; Leggett RE; Levin RM. 1992. Urinary bladder function in the tight-skin mouse. J Urol 148(5):1611-4. [PubMed: 1433576]  [MGI Ref ID J:3616]

Marie I; Beny JL. 2002. Endothelial dysfunction in murine model of systemic sclerosis: tight-skin mice 1. J Invest Dermatol 119(6):1379-87. [PubMed: 12485443]  [MGI Ref ID J:81020]

Martorana PA; van Even P; Gardi C; Lungarella G. 1989. A 16-month study of the development of genetic emphysema in tight-skin mice. Am Rev Respir Dis 139(1):226-32. [PubMed: 2912343]  [MGI Ref ID J:152520]

Matsushita T; Fujimoto M; Hasegawa M; Matsushita Y; Komura K; Ogawa F; Watanabe R; Takehara K; Sato S. 2007. BAFF antagonist attenuates the development of skin fibrosis in tight-skin mice. J Invest Dermatol 127(12):2772-80. [PubMed: 17581616]  [MGI Ref ID J:127329]

Matsushita Y; Hasegawa M; Matsushita T; Fujimoto M; Horikawa M; Fujita T; Kawasuji A; Ogawa F; Steeber DA; Tedder TF; Takehara K; Sato S. 2007. Intercellular adhesion molecule-1 deficiency attenuates the development of skin fibrosis in tight-skin mice. J Immunol 179(1):698-707. [PubMed: 17579093]  [MGI Ref ID J:143152]

McGaha T; Saito S; Phelps RG; Gordon R; Noben-Trauth N; Paul WE; Bona C. 2001. Lack of skin fibrosis in tight skin (TSK) mice with targeted mutation in the interleukin-4R alpha and transforming growth factor-beta genes. J Invest Dermatol 116(1):136-43. [PubMed: 11168809]  [MGI Ref ID J:68448]

Menon RP; Menon MR; Shi-Wen X; Renzoni E; Bou-Gharios G; Black CM; Abraham DJ. 2006. Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: insight into the functional role of mutant fibrillin-1. Exp Cell Res 312(9):1463-74. [PubMed: 16488411]  [MGI Ref ID J:111492]

Menton DN; Hess RA. 1980. The ultrastructure of collagen in the dermis of tight-skin (Tsk) mutant mice. J Invest Dermatol 74(3):139-47. [PubMed: 7359004]  [MGI Ref ID J:6273]

Muryoi T; Andre-Schwartz J; Saitoh Y; Daian C; Hall B; Dimitriu-Bona A; Schwartz RS; Bona CA; Kasturi KN. 1992. Self-reactive repertoire of tight skin (TSK/+) mouse: immunochemical and molecular characterization of anti-cellular autoantibodies. Cell Immunol 144(1):43-54. [PubMed: 1382866]  [MGI Ref ID J:2646]

O'dell NL; Burlison SK; Starcher BC; Pennington CB. 1996. Morphological and biochemical studies of the elastic fibres in the craniomandibular joint articular disc of the tight-skin mouse. Arch Oral Biol 41(5):431-7. [PubMed: 8809305]  [MGI Ref ID J:34650]

Omens JH; Rockman HA; Covell JW. 1994. Passive ventricular mechanics in tight-skin mice. Am J Physiol 266(3 Pt 2):H1169-76. [PubMed: 8160820]  [MGI Ref ID J:17498]

Ong CJ; Ip S; Teh SJ; Wong C; Jirik FR; Grusby MJ; Teh HS. 1999. A role for T helper 2 cells in mediating skin fibrosis in tight-skin mice. Cell Immunol 196(1):60-8. [PubMed: 10486156]  [MGI Ref ID J:57957]

Ong VH; Evans LA; Shiwen X; Fisher IB; Rajkumar V; Abraham DJ; Black CM; Denton CP. 2003. Monocyte chemoattractant protein 3 as a mediator of fibrosis: Overexpression in systemic sclerosis and the type 1 tight-skin mouse. Arthritis Rheum 48(7):1979-91. [PubMed: 12847692]  [MGI Ref ID J:106169]

Pablos JL; Carreira PE; Serrano L; Del Castillo P; Gomez-Reino JJ. 1997. Apoptosis and proliferation of fibroblasts during postnatal skin development and scleroderma in the tight-skin mouse. J Histochem Cytochem 45(5):711-9. [PubMed: 9154158]  [MGI Ref ID J:40179]

Pablos JL; Everett ET; Harley R; LeRoy EC; Norris JS. 1995. Transforming growth factor-beta 1 and collagen gene expression during postnatal skin development and fibrosis in the tight-skin mouse. Lab Invest 72(6):670-8. [PubMed: 7783425]  [MGI Ref ID J:26096]

Phelps RG; Daian C; Shibata S; Fleischmajer R; Bona CA. 1993. Induction of skin fibrosis and autoantibodies by infusion of immunocompetent cells from tight skin mice into C57BL/6 Pa/Pa mice. J Autoimmun 6(6):701-18. [PubMed: 8155252]  [MGI Ref ID J:16435]

Philips N; Bashey RI; Jimenez SA. 1995. Increased alpha 1(I) procollagen gene expression in tight skin (TSK) mice myocardial fibroblasts is due to a reduced interaction of a negative regulatory sequence with AP-1 transcription factor. J Biol Chem 270(16):9313-21. [PubMed: 7721853]  [MGI Ref ID J:24523]

Podowski M; Calvi CL; Cheadle C; Tuder RM; Biswals S; Neptune ER. 2009. Complex integration of matrix, oxidative stress, and apoptosis in genetic emphysema. Am J Pathol 175(1):84-96. [PubMed: 19541933]  [MGI Ref ID J:149984]

Ross SC; Osborn TG; Dorner RW; Zuckner J. 1983. Glycosaminoglycan content in skin of the tight-skin mouse. Arthritis Rheum 26(5):653-7. [PubMed: 6847727]  [MGI Ref ID J:7057]

Rossi GA; Hunninghake GW; Szapiel SV; Gadek JE; Fulmer JD; Kawanami O; Ferrans VJ; Crystal RG. 1980. The tight-skin mouse: an animal model of inherited emphysema. Bull Eur Physiopathol Respir 16 Suppl:157-66. [PubMed: 6971670]  [MGI Ref ID J:30961]

Saito E; Fujimoto M; Hasegawa M; Komura K; Hamaguchi Y; Kaburagi Y; Nagaoka T; Takehara K; Tedder TF; Sato S. 2002. CD19-dependent B lymphocyte signaling thresholds influence skin fibrosis and autoimmunity in the tight-skin mouse. J Clin Invest 109(11):1453-62. [PubMed: 12045259]  [MGI Ref ID J:76812]

Saito S; Nishimura H; Brumeanu TD; Casares S; Stan AC; Honjo T ; Bona CA. 1999. Characterization of mutated protein encoded by partially duplicated fibrillin-1 gene in tight skin (TSK) mice. Mol Immunol 36(3):169-76. [PubMed: 10403482]  [MGI Ref ID J:55886]

Sgonc R; Dietrich H; Sieberer C; Wick G; Christner PJ; Jimenez SA. 1999. Lack of endothelial cell apoptosis in the dermis of tight skin 1 and tight skin 2 mice. Arthritis Rheum 42(3):581-4. [PubMed: 10088787]  [MGI Ref ID J:55591]

Shen Y; Ichino M; Nakazawa M; Minami M. 2005. CpG oligodeoxynucleotides prevent the development of scleroderma-like syndrome in tight-skin mice by stimulating a Th1 immune response. J Invest Dermatol 124(6):1141-8. [PubMed: 15955088]  [MGI Ref ID J:98858]

Shibata S; Muryoi T; Saitoh Y; Brumeanu TD; Bona CA; Kasturi KN. 1993. Immunochemical and molecular characterization of anti-RNA polymerase I autoantibodies produced by tight skin mouse. J Clin Invest 92(2):984-92. [PubMed: 8349828]  [MGI Ref ID J:14182]

Siracusa LD; McGrath R; Fisher JK; Jimenez SA. 1998. The mouse tight skin (Tsk) phenotype is not dependent on the presence of mature T and B lymphocytes. Mamm Genome 9(11):907-9. [PubMed: 9799844]  [MGI Ref ID J:50581]

Sundberg JP (ed.). 1994. Handbook of Mouse Mutations with Skin and Hair Abnormalities: Animal Models and Biomedical Tools. In: Handbook of Mouse Mutations with Skin and Hair Abnormalities: Animal Models and Biomedical Tools. CRC Press, Boca Raton.  [MGI Ref ID J:30359]

Swank RT; Reddington M; Howlett O; Novak EK. 1991. Platelet storage pool deficiency associated with inherited abnormalities of the inner ear in the mouse pigment mutants muted and mocha. Blood 78(8):2036-44. [PubMed: 1912584]  [MGI Ref ID J:29151]

Tsuji-Yamada J; Nakazawa M; Takahashi K; Iijima K; Hattori S; Okuda K; Minami M; Ikezawa Z; Sasaki T. 2001. Effect of IL-12 encoding plasmid administration on tight-skin mouse. Biochem Biophys Res Commun 280(3):707-12. [PubMed: 11162578]  [MGI Ref ID J:67087]

Walker M; Harley R; Maize J; DeLustro F; LeRoy EC. 1985. Mast cells and their degranulation in the Tsk mouse model of scleroderma. Proc Soc Exp Biol Med 180(2):323-8. [PubMed: 4048170]  [MGI Ref ID J:8047]

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Weihrauch D; Xu H; Shi Y; Wang J; Brien J; Jones DW; Kaul S; Komorowski RA; Csuka ME; Oldham KT; Pritchard KA. 2007. Effects of D-4F on vasodilation, oxidative stress, angiostatin, myocardial inflammation, and angiogenic potential in tight-skin mice. Am J Physiol Heart Circ Physiol 293(3):H1432-41. [PubMed: 17496220]  [MGI Ref ID J:126106]

Xu H; Zaidi M; Struve J; Jones DW; Krolikowski JG; Nandedkar S; Lohr NL; Gadicherla A; Pagel PS; Csuka ME; Pritchard KA; Weihrauch D. 2011. Abnormal fibrillin-1 expression and chronic oxidative stress mediate endothelial mesenchymal transition in a murine model of systemic sclerosis. Am J Physiol Cell Physiol 300(3):C550-6. [PubMed: 21160034]  [MGI Ref ID J:171173]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB27

Colony Maintenance

Breeding & HusbandryHomozygotes are not viable. When maintaining a live colony, C57BL/6J female mice may be bred with heterozygous male mice.
Mating SystemInbred x Heterozygote         (Female x Male)   11-DEC-12
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $195.00Female or MaleHeterozygous for Fbn1Tsk  
Price per Pair (US dollars $)Pair Genotype
$216.25C57BL/6J (000664) x Heterozygous for Fbn1Tsk  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $253.50Female or MaleHeterozygous for Fbn1Tsk  
Price per Pair (US dollars $)Pair Genotype
$281.20C57BL/6J (000664) x Heterozygous for Fbn1Tsk  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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