Strain Name:

B6D2-Sox18Ra-Op/J

Stock Number:

000508

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Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names B6D2-Sox18Ra-op/J    (Changed: 05-JUL-05 )
Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationN77p
Generation Definitions

Description
The Sox18Ra-Op allele causes a more severe phenotype than either the Sox18Ra or Sox18Ra-J allele. Green and Mann determined that only approximately 17% of the Sox18Ra-Op/+ heterozygotes are born and survive to wean age. Heterozygotes have a deficiency of vibrissae, a distinguishing feature in utero and usually evident at birth, and can be distinguished at three days of age by their pink skin which, with its delayed coat development, fails to darken like that of wildtype siblings. A paucity of fur is apparent by 9 days of age and persists throughout life. Heterozygotes are smaller in overall size and many of those that die shortly after birth are cyanotic and edematous. Dead pups have glossy skin with red patches that may result from superficial hemorrhages. Homozygotes die by embryonic day 11.5. (Green and Mann, 1961; Mann, 1963.)

Related Strains

Strains carrying other alleles of Sox18
000018   B6.Cg-Sox18Ra/J
010987   B6;129S-Sox18tm1(GFP/cre/ERT2)Pzg/J
000125   B6By.Cg-Sox18Ra Pt Os/J
023020   B6N(Cg)-Sox18tm1.1(KOMP)Vlcg/J
002261   C3H/HeSnJ-Sox18Ra-J/J
000267   ROP/GnLeJ
View Strains carrying other alleles of Sox18     (6 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).
Hypotrichosis-Lymphedema-Telangiectasia Syndrome; HLTS
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Sox18Ra-Op/Sox18+

        involves: C57BL/6J * DBA/2J
  • mortality/aging
  • partial perinatal lethality
    • many mice are dead or dying shortly after birth   (MGI Ref ID J:260)
    • many mice are dead or dying at birth   (MGI Ref ID J:279)
  • partial postnatal lethality
    • fewer than expected mice are present at weaning   (MGI Ref ID J:260)
    • edematous mice die prior to weaning   (MGI Ref ID J:279)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:279)
    • mice are smaller than normal from birth up to three months of age   (MGI Ref ID J:260)
    • at weaning mice are two-thirds the size of wild-type mice   (MGI Ref ID J:260)
  • distended abdomen
    • mice that die prior to weaning exhibit swollen abdomens with perintoneal cavities filled with thin, white liquid unlike in wild-type mice   (MGI Ref ID J:279)
  • homeostasis/metabolism phenotype
  • cyanosis
    • in some mice at birth   (MGI Ref ID J:260)
  • edema   (MGI Ref ID J:279)
    • at birth, some mice are edematous   (MGI Ref ID J:260)
    • mice that die around birth are edematous in the neck, shoulders, and abdominal and pleural cavities   (MGI Ref ID J:260)
    • skin edema
      • mice that die around birth have edematous skin that is stretched, glossy, membranous in appearance, with red patches   (MGI Ref ID J:260)
  • integument phenotype
  • abnormal awl hair morphology
    • mice have some awl hairs unlike in wild-type mice   (MGI Ref ID J:260)
  • abnormal hair follicle morphology
    • mice lack follicles in the quiescent stage and are abnormal with bulbs that are twisted and curved unlike in wild-type mice   (MGI Ref ID J:279)
  • abnormal hair growth
    • asynchronous   (MGI Ref ID J:260)
    • asynchronous   (MGI Ref ID J:279)
    • delayed hair appearance
      • at 3 days of age, mice are still pink due to sparse coat unlike wild-type mice that have developed a coat   (MGI Ref ID J:260)
      • initiation of each follicle type is delayed compared to in wild-type mice   (MGI Ref ID J:279)
    • sparse hair   (MGI Ref ID J:260)
  • abnormal vibrissa morphology
    • at E17, mystacial vibrissa are incomplete and post-orbital vibrissa are absent or reduced in size compared to in wild-type mice   (MGI Ref ID J:279)
    • mice lack inter-ramal and anterior supra-orbital vibrissa unlike wild-type mice   (MGI Ref ID J:279)
    • postoral and posterior supra-orbital vibrassa are decreased in number compared to in wild-type mice   (MGI Ref ID J:279)
    • abnormal vibrissa number
      • anterior vibrissae and their follicles are absent unlike in wild-type mice   (MGI Ref ID J:260)
    • short vibrissae
      • at E17, post-orbital vibrissa are shorter than normal   (MGI Ref ID J:279)
  • abnormal zigzag hair morphology
    • some zigzag hairs are narrow, curled, lacked constrictions, and contain small medullar cells which lack pigment unlike in wild-type mice   (MGI Ref ID J:279)
    • decreased zigzag hair amount
      • only a small patch on the shoulders   (MGI Ref ID J:260)
      • zigzag hairs are absent in the middle of the dorsum   (MGI Ref ID J:279)
  • absent auchene hairs   (MGI Ref ID J:260)
  • skin edema
    • mice that die around birth have edematous skin that is stretched, glossy, membranous in appearance, with red patches   (MGI Ref ID J:260)

Sox18Ra-Op/Sox18Ra-Op

        involves: C57BL/6J * DBA/2J
  • mortality/aging
  • complete embryonic lethality
    • mice die at E11 or earlier   (MGI Ref ID J:279)
  • complete preweaning lethality
    • no mice are found (no time of observation provided)   (MGI Ref ID J:260)

Sox18Ra-Op/Sox18Ra-Op

        B6D2-Sox18Ra-Op/J
  • mortality/aging
  • complete lethality throughout fetal growth and development
    • mice die before E14.5   (MGI Ref ID J:143564)
    • mice do not survive beyond E14.5   (MGI Ref ID J:150211)
  • cardiovascular system phenotype
  • *normal* cardiovascular system phenotype
    • between E8.5 and E10.5, differentiation, proliferation, migration and association of endothelial cells, and patterning and establishment of the primary vascular plexus is normal   (MGI Ref ID J:150211)
    • abnormal blood vessel morphology
      • at E12.5, 67% of mice exhibit enlarged surface microvasculature in the trunk, head, proximal limb and neck unlike in wild-type mice   (MGI Ref ID J:150211)
      • subdermal microvasculature diameter is increased 21% at E12.5 and 35% at E13.5 compared to in wild-type mice   (MGI Ref ID J:150211)
      • vascular defects increase in severity with age   (MGI Ref ID J:150211)
      • at E13.5, all mice exhibit enlarged surface microvasculature compared to in wild-type mice   (MGI Ref ID J:150211)
      • at E14.5, surface microvessels, particularly around the anterior cranial region, are enlarged compared to in wild-type mice   (MGI Ref ID J:150211)
      • enlarged blood vessels frequently rupture   (MGI Ref ID J:150211)
      • abnormal pericyte morphology
        • fewer pericytes are recruited to microvasculature compared to in wild-type mice   (MGI Ref ID J:150211)
      • increased vascular endothelial cell number
        • endothelial hyperplasia is associated with defects in pericyte recruitment   (MGI Ref ID J:150211)
    • abnormal vascular endothelial cell physiology
      • loss of vascular integrity is due to endothelial rupture   (MGI Ref ID J:150211)
    • hemorrhage
      • at E13.5, mice exhibit multiple regions of vascular hemorrhage unlike in wild-type mice   (MGI Ref ID J:150211)
      • at E14.5, large-scale vascular hemorrhage is observed in all mice   (MGI Ref ID J:150211)
      • enlarged blood vessels frequently rupture   (MGI Ref ID J:150211)
      • hematoma
        • over large areas of the trunk, neck, and head   (MGI Ref ID J:150211)
  • homeostasis/metabolism phenotype
  • edema
    • generalized at E13.5   (MGI Ref ID J:150211)
    • skin edema
      • at E13.5   (MGI Ref ID J:143564)
      • 63% of mice exhibit severe subcutaneous edema in the trunk and neck regions un like in wild-type mice   (MGI Ref ID J:150211)
  • immune system phenotype
  • abnormal lymphatic vessel morphology
    • lymphatic vessels are finer, denser, and more branched with more loops and fewer blind-ended sacs compared to in wild-type mice   (MGI Ref ID J:143564)
  • integument phenotype
  • skin edema
    • at E13.5   (MGI Ref ID J:143564)
    • 63% of mice exhibit severe subcutaneous edema in the trunk and neck regions un like in wild-type mice   (MGI Ref ID J:150211)
View Research Applications

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

Sox18Ra-Op related

Dermatology Research
Skin and Hair Texture Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Sox18Ra-Op
Allele Name opossum
Allele Type Spontaneous
Common Name(s) Op; RaOp;
Strain of Origin(C57BL/6J x DBA/2J)F1
Gene Symbol and Name Sox18, SRY (sex determining region Y)-box 18
Chromosome 2
Gene Common Name(s) AI385749; HLTS; Ra; Ragl; Sry-related HMG-box gene 18; expressed sequence AI385749; ragged; ragged-like;
Molecular Note The mutation was identified as a deletion of a single cytosine at nucleotide 1048. [MGI Ref ID J:74211]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Dunn TL; Mynett-Johnson L; Wright EM; Hosking BM; Koopman PA; Muscat GE. 1995. Sequence and expression of Sox-18 encoding a new HMG-box transcription factor. Gene 161(2):223-5. [PubMed: 7665083]  [MGI Ref ID J:28369]

Hosking BM; Wyeth JR; Pennisi DJ; Wang SC; Koopman P; Muscat GE. 2001. Cloning and functional analysis of the Sry-related HMG box gene, Sox18. Gene 262(1-2):239-47. [PubMed: 11179689]  [MGI Ref ID J:67559]

James K; Hosking B; Gardner J; Muscat GE; Koopman P. 2003. Sox18 mutations in the ragged mouse alleles ragged-like and opossum. Genesis 36(1):1-6. [PubMed: 12748961]  [MGI Ref ID J:83731]

Pennisi D; Bowles J; Nagy A; Muscat G; Koopman P. 2000. Mice null for sox18 are viable and display a mild coat defect Mol Cell Biol 20(24):9331-6. [PubMed: 11094083]  [MGI Ref ID J:66010]

Pennisi D; Gardner J; Chambers D; Hosking B; Peters J; Muscat G; Abbott C; Koopman P. 2000. Mutations in Sox18 underlie cardiovascular and hair follicle defects in ragged mice. Nat Genet 24(4):434-7. [PubMed: 10742113]  [MGI Ref ID J:61488]

Sox18Ra-Op related

Brouillard P; Boon L; Vikkula M. 2014. Genetics of lymphatic anomalies. J Clin Invest 124(3):898-904. [PubMed: 24590274]  [MGI Ref ID J:209628]

Downes M; Francois M; Ferguson C; Parton RG; Koopman P. 2009. Vascular defects in a mouse model of hypotrichosis-lymphedema-telangiectasia syndrome indicate a role for SOX18 in blood vessel maturation. Hum Mol Genet 18(15):2839-50. [PubMed: 19429912]  [MGI Ref ID J:150211]

Downes M; Koopman P. 2001. SOX18 and the transcriptional regulation of blood vessel development. Trends Cardiovasc Med 11(8):318-24. [PubMed: 11728880]  [MGI Ref ID J:74211]

Francois M; Caprini A; Hosking B; Orsenigo F; Wilhelm D; Browne C; Paavonen K; Karnezis T; Shayan R; Downes M; Davidson T; Tutt D; Cheah KS; Stacker SA; Muscat GE; Achen MG; Dejana E; Koopman P. 2008. Sox18 induces development of the lymphatic vasculature in mice. Nature 456(7222):643-7. [PubMed: 18931657]  [MGI Ref ID J:143564]

Green EL; Mann SJ. 1961. Opossum, a semi-dominant lethal mutation affecting hair and other characteristics of mice J Hered 52:223-7.  [MGI Ref ID J:260]

James K; Hosking B; Gardner J; Muscat GE; Koopman P. 2003. Sox18 mutations in the ragged mouse alleles ragged-like and opossum. Genesis 36(1):1-6. [PubMed: 12748961]  [MGI Ref ID J:83731]

Kartopawiro J; Bower NI; Karnezis T; Kazenwadel J; Betterman KL; Lesieur E; Koltowska K; Astin J; Crosier P; Vermeren S; Achen MG; Stacker SA; Smith KA; Harvey NL; Francois M; Hogan BM. 2014. Arap3 is dysregulated in a mouse model of hypotrichosis-lymphedema-telangiectasia and regulates lymphatic vascular development. Hum Mol Genet 23(5):1286-97. [PubMed: 24163130]  [MGI Ref ID J:206218]

Mann S. 1962. Prenatal formation of hair follicle types Anat Rec 144:135-142.  [MGI Ref ID J:155792]

Mann SJ. 1963. The phenogenetics of hair mutants in the house mouse: Opossum and Ragged Genet Res 4:1-11.  [MGI Ref ID J:279]

Oommen S; Francois M; Kawasaki M; Murrell M; Kawasaki K; Porntaveetus T; Ghafoor S; Young NJ; Okamatsu Y; McGrath J; Koopman P; Sharpe PT; Ohazama A. 2012. Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function. PLoS One 7(9):e43857. [PubMed: 22962592]  [MGI Ref ID J:191641]

Pennisi D; Gardner J; Chambers D; Hosking B; Peters J; Muscat G; Abbott C; Koopman P. 2000. Mutations in Sox18 underlie cardiovascular and hair follicle defects in ragged mice. Nat Genet 24(4):434-7. [PubMed: 10742113]  [MGI Ref ID J:61488]

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]

Wallace ME. 1966. Chylous ascites pleiotropic effect of Ra. Mouse News Lett 35:16.  [MGI Ref ID J:13448]

Young N; Hahn CN; Poh A; Dong C; Wilhelm D; Olsson J; Muscat GE; Parsons P; Gamble JR; Koopman P. 2006. Effect of disrupted SOX18 transcription factor function on tumor growth, vascularization, and endothelial development. J Natl Cancer Inst 98(15):1060-7. [PubMed: 16882943]  [MGI Ref ID J:112009]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3300.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4290.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

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