Strain Name:

B6.129-Notch1tm1Con/J

Stock Number:

002797

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Availability:

Cryopreserved - Ready for recovery

Somitogenesis is disturbed in these Notch1 deficient mice.

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

Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain 129X1 x 129S1 via R1-S3 (+Kitl-SlJ) ES cell line
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Appearance
black
Related Genotype: a/a

Description
In these Notch1 deficient mice, somitogenesis is disturbed and there is a probable defect in chorio-allantoic connection. Homozygotes die at embryonic day 9.

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 mutant was generated by deleting a large portion of the Notch1 gene using a positive/negative targeting vector. These mice have been backcrossed to C57BL/6J mice (Stock No. 000664) for at least 10 generations.

Control Information

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

Related Strains

Strains carrying   Notch1tm1Con allele
002445   STOCK Notch1tm1Con/J
View Strains carrying   Notch1tm1Con     (1 strain)

Strains carrying other alleles of Notch1
007181   B6.129X1-Notch1tm2Rko/GridJ
003332   C57BL/6-Tg(LckNotch1)9Erob/J
008159   STOCK Gt(ROSA)26Sortm1(Notch1)Dam/J
006951   STOCK Notch1tm2Rko/GridJ
006953   STOCK Notch1tm3(cre)Rko/J
View Strains carrying other alleles of Notch1     (5 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Aortic Valve Disease 1; AOVD1   (NOTCH1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Notch1tm1Con/Notch1tm1Con

        either: (involves: 129S1/Sv * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6) or (involves: 129S1/Sv * 129X1/SvJ * CD-1)
  • mortality/aging
  • complete embryonic lethality during organogenesis
    • most mutants are dead at E10 and all die by E11   (MGI Ref ID J:25248)
  • embryogenesis phenotype
  • abnormal rostral-caudal axis patterning
    • show a deficit in posterior development at E9   (MGI Ref ID J:25248)
  • abnormal somite development   (MGI Ref ID J:62882)
    • mutants exhibit a lack of coordination in segmentation of the somites, leading to variable somite size and misalignment of the somites across the midline of the embryo   (MGI Ref ID J:25248)
    • transition from presomitic mesoderm to the somite is disordered   (MGI Ref ID J:25248)
    • the most recently formed somites are not as tightly packed as those of wild-type and the epithelialization sometimes appears incomplete   (MGI Ref ID J:25248)
    • abnormal left-right axis symmetry of the somites
      • lack of coordination across the midline in the segmentation of somites, where segmentation is sometimes present on one side but not the other   (MGI Ref ID J:25248)
    • abnormal somite size
      • variable somite size   (MGI Ref ID J:25248)
    • delayed somite formation
      • mutants exhibit a delay in segmentation of the somites   (MGI Ref ID J:25248)
  • decreased embryo size
    • embryos are smaller at E9   (MGI Ref ID J:25248)
  • embryonic growth arrest   (MGI Ref ID J:62882)
    • growth arrest at the 14 somite stage   (MGI Ref ID J:25248)
  • kinked neural tube
    • neural tube kinks are seen extending posterior to the forelimb   (MGI Ref ID J:62882)
  • notochord degeneration
    • the notochord degenerates after growth arrest   (MGI Ref ID J:25248)
  • growth/size/body phenotype
  • decreased embryo size
    • embryos are smaller at E9   (MGI Ref ID J:25248)
  • cardiovascular system phenotype
  • distended pericardium   (MGI Ref ID J:62882)
    • distended pericardia at E9   (MGI Ref ID J:25248)
  • cellular phenotype
  • abnormal cell death
    • an increase in cell death is seen in embryos after they arrest   (MGI Ref ID J:25248)
  • nervous system phenotype
  • kinked neural tube
    • neural tube kinks are seen extending posterior to the forelimb   (MGI Ref ID J:62882)

Notch1tm1Con/Notch1tm1Con

        involves: 129S1/Sv * 129X1/SvJ
  • cardiovascular system phenotype
  • abnormal myocardium layer morphology
    • the endocardium does not surround the myocardium, which shows irregular thickness   (MGI Ref ID J:119151)
    • disorganized myocardium
      • less-structured myocardium   (MGI Ref ID J:119151)
    • trabecula carnea hypoplasia   (MGI Ref ID J:119151)
  • muscle phenotype
  • trabecula carnea hypoplasia   (MGI Ref ID J:119151)
View Research Applications

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

Notch1tm1Con related

Developmental Biology Research
Embryonic Lethality (Homozygous)

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Notch1tm1Con
Allele Name targeted mutation 1, Ronald L Conlon
Allele Type Targeted (knock-out)
Common Name(s) N1delta1; Notch1-; Notch1tm1Con/J; Notch-; Notchdelta1;
Mutation Made ByDr. Ronald Conlon,   Case Western Reserve Univ, School of Med
Strain of Origin(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
ES Cell Line NameR1
ES Cell Line Strain(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Gene Symbol and Name Notch1, notch 1
Chromosome 2
Gene Common Name(s) 9930111A19Rik; Mis6; Motch A; N1; NOTCH; RIKEN cDNA 9930111A19 gene; TAN1; Tan1; hN1; lin-12; translocation-associated Notch;
Molecular Note Deletion of sequences encoding the EGF repeats 28 to 36, three Notch repeats, the transmembrane domain and CDC10/SWI6 repeats, and replacement with a neomycin gene. [MGI Ref ID J:25248]

Genotyping

Genotyping Information

Genotyping Protocols

Notch1tm1Con, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Conlon RA; Reaume AG; Rossant J. 1995. Notch1 is required for the coordinate segmentation of somites. Development 121(5):1533-45. [PubMed: 7789282]  [MGI Ref ID J:25248]

Additional References

Notch1tm1Con related

Barrantes IB; Elia AJ; Wunsch K; De Angelis MH; Mak TW; Rossant J; Conlon RA; Gossler A; de la Pompa JL. 1999. Interaction between Notch signalling and Lunatic fringe during somite boundary formation in the mouse. Curr Biol 9(9):470-80. [PubMed: 10330372]  [MGI Ref ID J:54606]

Cheng HT; Kim M; Valerius MT; Surendran K; Schuster-Gossler K; Gossler A; McMahon AP; Kopan R. 2007. Notch2, but not Notch1, is required for proximal fate acquisition in the mammalian nephron. Development 134(4):801-11. [PubMed: 17229764]  [MGI Ref ID J:119907]

Dale JK; Malapert P; Chal J; Vilhais-Neto G; Maroto M; Johnson T; Jayasinghe S; Trainor P; Herrmann B; Pourquie O. 2006. Oscillations of the snail genes in the presomitic mesoderm coordinate segmental patterning and morphogenesis in vertebrate somitogenesis. Dev Cell 10(3):355-66. [PubMed: 16516838]  [MGI Ref ID J:106622]

Ge C; Stanley P. 2010. Effects of varying Notch1 signal strength on embryogenesis and vasculogenesis in compound mutant heterozygotes. BMC Dev Biol 10:36. [PubMed: 20346184]  [MGI Ref ID J:160476]

Girard L; Jolicoeur P. 1998. A full-length Notch1 allele is dispensable for transformation associated with a provirally activated truncated Notch1 allele in Moloney MuLV-infected MMTV(D)/myc transgenic mice. Oncogene 16(4):517-22. [PubMed: 9484841]  [MGI Ref ID J:45669]

Grego-Bessa J; Luna-Zurita L; del Monte G; Bolos V; Melgar P; Arandilla A; Garratt AN; Zang H; Mukouyama YS; Chen H; Shou W; Ballestar E; Esteller M; Rojas A; Perez-Pomares JM; de la Pompa JL. 2007. Notch signaling is essential for ventricular chamber development. Dev Cell 12(3):415-29. [PubMed: 17336907]  [MGI Ref ID J:119151]

Hadland BK; Huppert SS; Kanungo J; Xue Y; Jiang R; Gridley T; Conlon RA; Cheng AM; Kopan R; Longmore GD. 2004. A requirement for Notch1 distinguishes 2 phases of definitive hematopoiesis during development. Blood 104(10):3097-105. [PubMed: 15251982]  [MGI Ref ID J:94898]

Hitoshi S; Alexson T; Tropepe V; Donoviel D; Elia AJ; Nye JS; Conlon RA; Mak TW; Bernstein A; van Der Kooy D. 2002. Notch pathway molecules are essential for the maintenance, but not the generation, of mammalian neural stem cells. Genes Dev 16(7):846-58. [PubMed: 11937492]  [MGI Ref ID J:75878]

Huppert SS; Ilagan MX; De Strooper B; Kopan R. 2005. Analysis of Notch Function in Presomitic Mesoderm Suggests a gamma-Secretase-Independent Role for Presenilins in Somite Differentiation. Dev Cell 8(5):677-88. [PubMed: 15866159]  [MGI Ref ID J:98438]

Huppert SS; Le A; Schroeter EH; Mumm JS; Saxena MT; Milner LA; Kopan R. 2000. Embryonic lethality in mice homozygous for a processing-deficient allele of Notch1. Nature 405(6789):966-70. [PubMed: 10879540]  [MGI Ref ID J:62882]

Kikuchi R; Takeshita K; Uchida Y; Kondo M; Cheng XW; Nakayama T; Yamamoto K; Matsushita T; Liao JK; Murohara T. 2011. Pitavastatin-induced angiogenesis and arteriogenesis is mediated by Notch1 in a murine hindlimb ischemia model without induction of VEGF. Lab Invest 91(5):691-703. [PubMed: 21301413]  [MGI Ref ID J:171259]

Kim YH; Hu H; Guevara-Gallardo S; Lam MT; Fong SY; Wang RA. 2008. Artery and vein size is balanced by Notch and ephrin B2/EphB4 during angiogenesis. Development 135(22):3755-64. [PubMed: 18952909]  [MGI Ref ID J:144857]

Kumano K; Chiba S; Kunisato A; Sata M; Saito T; Nakagami-Yamaguchi E; Yamaguchi T; Masuda S; Shimizu K; Takahashi T; Ogawa S; Hamada Y; Hirai H. 2003. Notch1 but not Notch2 is essential for generating hematopoietic stem cells from endothelial cells. Immunity 18(5):699-711. [PubMed: 12753746]  [MGI Ref ID J:83475]

Li T; Wen H; Brayton C; Das P; Smithson LA; Fauq A; Fan X; Crain BJ; Price DL; Golde TE; Eberhart CG; Wong PC. 2007. Epidermal growth factor receptor and notch pathways participate in the tumor suppressor function of gamma-secretase. J Biol Chem 282(44):32264-73. [PubMed: 17827153]  [MGI Ref ID J:126817]

Liu Z; Chen S; Boyle S; Zhu Y; Zhang A; Piwnica-Worms DR; Ilagan MX; Kopan R. 2013. The Extracellular Domain of Notch2 Increases Its Cell-Surface Abundance and Ligand Responsiveness during Kidney Development. Dev Cell 25(6):585-98. [PubMed: 23806616]  [MGI Ref ID J:198632]

Liu Z; Obenauf AC; Speicher MR; Kopan R. 2009. Rapid identification of homologous recombinants and determination of gene copy number with reference/query pyrosequencing (RQPS). Genome Res 19(11):2081-9. [PubMed: 19797679]  [MGI Ref ID J:172930]

Loomes KM; Stevens SA; O'brien ML; Gonzalez DM; Ryan MJ; Segalov M; Dormans NJ; Mimoto MS; Gibson JD; Sewell W; Schaffer AA; Nah HD; Rappaport EF; Pratt SC; Dunwoodie SL; Kusumi K. 2007. Dll3 and Notch1 genetic interactions model axial segmental and craniofacial malformations of human birth defects. Dev Dyn 236(10):2943-51. [PubMed: 17849441]  [MGI Ref ID J:125511]

Nakagawa M; Ichikawa M; Kumano K; Goyama S; Kawazu M; Asai T; Ogawa S; Kurokawa M; Chiba S. 2006. AML1/Runx1 rescues Notch1-null mutation-induced deficiency of para-aortic splanchnopleural hematopoiesis. Blood 108(10):3329-34. [PubMed: 16888092]  [MGI Ref ID J:140445]

Nus M; MacGrogan D; Martinez-Poveda B; Benito Y; Casanova JC; Fernandez-Aviles F; Bermejo J; de la Pompa JL. 2011. Diet-induced aortic valve disease in mice haploinsufficient for the Notch pathway effector RBPJK/CSL. Arterioscler Thromb Vasc Biol 31(7):1580-8. [PubMed: 21493891]  [MGI Ref ID J:187551]

Takeshita K; Satoh M; Ii M; Silver M; Limbourg FP; Mukai Y; Rikitake Y; Radtke F; Gridley T; Losordo DW; Liao JK. 2007. Critical role of endothelial Notch1 signaling in postnatal angiogenesis. Circ Res 100(1):70-8. [PubMed: 17158336]  [MGI Ref ID J:130243]

Tan JB; Visan I; Yuan JS; Guidos CJ. 2005. Requirement for Notch1 signals at sequential early stages of intrathymic T cell development. Nat Immunol 6(7):671-9. [PubMed: 15951812]  [MGI Ref ID J:99151]

Thomas JA; Haudek SB; Koroglu T; Tsen MF; Bryant DD; White DJ; Kusewitt DF; Horton JW; Giroir BP. 2003. IRAK1 deletion disrupts cardiac Toll/IL-1 signaling and protects against contractile dysfunction. Am J Physiol Heart Circ Physiol 285(2):H597-606. [PubMed: 12860565]  [MGI Ref ID J:84829]

Timmerman LA; Grego-Bessa J; Raya A; Bertran E; Perez-Pomares JM; Diez J; Aranda S; Palomo S; McCormick F; Izpisua-Belmonte JC; de la Pompa JL. 2004. Notch promotes epithelial-mesenchymal transition during cardiac development and oncogenic transformation. Genes Dev 18(1):99-115. [PubMed: 14701881]  [MGI Ref ID J:87765]

Visan I; Tan JB; Yuan JS; Harper JA; Koch U; Guidos CJ. 2006. Regulation of T lymphopoiesis by Notch1 and Lunatic fringe-mediated competition for intrathymic niches. Nat Immunol 7(6):634-43. [PubMed: 16699526]  [MGI Ref ID J:112675]

de la Pompa JL; Wakeham A; Correia KM; Samper E; Brown S; Aguilera RJ ; Nakano T ; Honjo T ; Mak TW ; Rossant J ; Conlon RA. 1997. Conservation of the Notch signalling pathway in mammalian neurogenesis. Development 124(6):1139-48. [PubMed: 9102301]  [MGI Ref ID J:39351]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryThis strain is maintained by breeding heterozygous mice to normal wildtype siblings or to C57BL/6J inbred mice (Stock No. 000664). Homozygotes die at embryonic day 9.

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* $2085.00
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 11 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* $2710.50
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 11 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.

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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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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