Strain Name:

B6;129-Ppt1tm1Hof/J

Stock Number:

004313

Order this mouse

Availability:

Cryopreserved - Ready for recovery

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 B6;129-Ppttm1Hof/J    (Changed: 15-DEC-04 )
Type Mutant Stock; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating Investigator Sandra Hofmann,   Center at Dallas

Description
Mice that are homozygous for the targeted mutation are viable, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (protein) is detected by immunoassay. Palmitoyl-protein thioesterase activity in the brain of the mutant is reduced to background levels. Although healthy at birth, by age four to five months, mutant mice display lack of grooming and an abnormal gait that progresses to hind limb paralysis. By six to eight months of age mutant mice have a low body weight and display an abnormal clasping behavior. Aggressive behavior results in fighting and dermatitis due to bite wounds. By seven months of age, mortality is 50% with very few mice surviving beyond ten months of age. Myoclonic jerks and seizures manifest at age three to four months. Strong rapid hind limb seizures ("popcorn" seizures) that propel mice several feet also occur. Brain size of the mutant mice is reduced. Histologically, mutant brains show neuronal loss and apoptosis in the hippocampus, cerebral cortex and cerebellum. Abundant autofluorescent storage material, termed granular osmiophilic deposits (GROD), seen under electron microscopic examination of mutant mouse brain tissue is indistinguishable from the GROD observed in human infantile neuronal ceroid lipofuscinosis. This mutant mouse strain represents a model that may be useful in studies of neuronal ceroid lipofuscinoses, including infantile Batten disease.

Development
A targeting vector containing neomycin resistance and herpes simplex virus thymidine kinase genes was used to disrupt exon 9 of the targeted gene. The construct was electroporated into 129S6/SvEvTac derived SM1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting male chimeric animals were backcrossed to C57BL/6J mice.

Related Strains

Strains carrying   Ppt1tm1Hof allele
006566   B6.129S6-Ppt1tm1Hof/SopJ
View Strains carrying   Ppt1tm1Hof     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Ceroid Lipofuscinosis, Neuronal, 3; CLN3
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Ceroid Lipofuscinosis, Neuronal, 1; CLN1   (PPT1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Ppt1tm1Hof/Ppt1tm1Hof

        involves: 129S6/SvEvTac * C57BL/6J
  • mortality/aging
  • premature death
    • death by 10 months of age   (MGI Ref ID J:72931)
  • behavior/neurological phenotype
  • abnormal aggression-related behavior
    • bite wounds in colony evident   (MGI Ref ID J:72931)
  • abnormal gait
    • lowering of pelvis, splayed hind limbs, hunched posture, side-to-side wobbling, evident beginning at 4 - 5 months of age and progressing to hind limb paralysis   (MGI Ref ID J:72931)
  • decreased grooming behavior
    • beginning at 4 to 5 months of age   (MGI Ref ID J:72931)
  • hindlimb paralysis
    • end point of abnormal gait   (MGI Ref ID J:72931)
  • jerky movement   (MGI Ref ID J:72931)
  • myoclonus
    • evident beginning at 3 to 4 months of age   (MGI Ref ID J:72931)
  • craniofacial phenotype
  • abnormal neurocranium morphology
    • thickened   (MGI Ref ID J:72931)
  • muscle phenotype
  • myoclonus
    • evident beginning at 3 to 4 months of age   (MGI Ref ID J:72931)
  • skeleton phenotype
  • abnormal neurocranium morphology
    • thickened   (MGI Ref ID J:72931)
  • nervous system phenotype
  • abnormal brain morphology
    • autofluorescent deposits, granular osmiophilic deposits, found in cells throughout brain   (MGI Ref ID J:72931)
    • storage at 5 months of age found in cerebral cortex, cerebellar Purkinje and granular layers, hippocampus, thalamus and hypothalamus, dentate nucleus, pons, and some areas of the medulla   (MGI Ref ID J:72931)
    • Purkinje cell degeneration   (MGI Ref ID J:72931)
    • decreased brain size   (MGI Ref ID J:72931)
    • hippocampal neuron degeneration   (MGI Ref ID J:72931)
  • myoclonus
    • evident beginning at 3 to 4 months of age   (MGI Ref ID J:72931)
  • neurodegeneration
    • cerebral cortex neurodegeneration   (MGI Ref ID J:72931)
    • Purkinje cell degeneration   (MGI Ref ID J:72931)
    • hippocampal neuron degeneration   (MGI Ref ID J:72931)

Ppt1tm1Hof/Ppt1tm1Hof

        involves: 129P2/OlaHsd * 129S2/SvPas * 129S6/SvEvTac * C57BL/6
  • mortality/aging
  • premature death
    • mutants die by 35 weeks of age, with a median survival of 33 weeks   (MGI Ref ID J:177265)
  • nervous system phenotype
  • abnormal brain morphology
    • mutants exhibit a 2.6-fold increase in autofluorescent accumulation in the brain compared to wild-type mice   (MGI Ref ID J:177265)
    • abnormal visual cortex morphology
      • atrophy of the primary visual cortex   (MGI Ref ID J:177265)
    • brain atrophy
      • by 5 months of age, brain atrophy is apparent   (MGI Ref ID J:177265)
    • decreased brain weight
      • decrease in brain weight by 13.4% is seen by 6 months of age   (MGI Ref ID J:177265)
    • hippocampal neuron degeneration   (MGI Ref ID J:177265)
    • thin cerebral cortex
      • at 6 months of age, 21.6% decrease in cortical thickness   (MGI Ref ID J:177265)
  • astrocytosis
    • reactive astrocytosis   (MGI Ref ID J:177265)
  • brain inflammation
    • mutants exhibit immune cell infiltration in the brain, with an increase in CD45+ leukocytes, CD3+ T-cells, and CD68+ large monocytes   (MGI Ref ID J:177265)
  • neurodegeneration
    • mutants exhibit progressive neurodegeneration, with degenerating neurons within the corpus callosum, hippocampus, and thalamus at 5 months of age   (MGI Ref ID J:177265)
    • hippocampal neuron degeneration   (MGI Ref ID J:177265)
  • homeostasis/metabolism phenotype
  • abnormal cytokine level
    • mutants exhibit elevated cytokine and chemokine levels at 3 months of age   (MGI Ref ID J:177265)
    • fewer cytokines and chemokines are elevated in single mutant mice than in triple Ppt1, Gfap and Vim homozygotes   (MGI Ref ID J:177265)
  • immune system phenotype
  • abnormal cytokine level
    • mutants exhibit elevated cytokine and chemokine levels at 3 months of age   (MGI Ref ID J:177265)
    • fewer cytokines and chemokines are elevated in single mutant mice than in triple Ppt1, Gfap and Vim homozygotes   (MGI Ref ID J:177265)
  • brain inflammation
    • mutants exhibit immune cell infiltration in the brain, with an increase in CD45+ leukocytes, CD3+ T-cells, and CD68+ large monocytes   (MGI Ref ID J:177265)
View Research Applications

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

Ppt1tm1Hof related

Developmental Biology Research
Growth Defects
Internal/Organ Defects
      brain

Neurobiology Research
Behavioral and Learning Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Ppt1tm1Hof
Allele Name targeted mutation 1, Sandra L Hoffmann
Allele Type Targeted (Null/Knockout)
Common Name(s) Cln1 mut; PPT1-; PPT1-KO;
Mutation Made By Praveena Gupta,  
Strain of Origin129S6/SvEvTac
ES Cell Line NameSM1
ES Cell Line Strain129S6/SvEvTac
Gene Symbol and Name Ppt1, palmitoyl-protein thioesterase 1
Chromosome 4
Gene Common Name(s) 9530043G02Rik; AA960502; C77813; CLN1; D4Ertd184e; DNA segment, Chr 4, ERATO Doi 184, expressed; INCL; PPT; RIKEN cDNA 9530043G02 gene; expressed sequence AA960502; expressed sequence C77813;
Molecular Note Part of exon 9 was replaced with a neomycin selection cassette containing an in frame stop codon that is predicted to cause premature termination of the protein. Nothern blot analysis on RNA derived from brain and other tissues of heterozygous and homozygous mice demonstrated that a truncated transcript is produced from this allele. However, western blot analysis and activity assays confirmed that no functional protein is expressed from this allele in homozygous mice. [MGI Ref ID J:72931]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Gupta P; Soyombo AA; Atashband A; Wisniewski KE; Shelton JM; Richardson JA; Hammer RE; Hofmann SL. 2001. Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice. Proc Natl Acad Sci U S A 98(24):13566-71. [PubMed: 11717424]  [MGI Ref ID J:72931]

Additional References

Ppt1tm1Hof related

Bible E; Gupta P; Hofmann SL; Cooper JD. 2004. Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis 16(2):346-59. [PubMed: 15193291]  [MGI Ref ID J:91134]

Bond M; Holthaus SM; Tammen I; Tear G; Russell C. 2013. Use of model organisms for the study of neuronal ceroid lipofuscinosis. Biochim Biophys Acta 1832(11):1842-65. [PubMed: 23338040]  [MGI Ref ID J:202442]

Cho SK; Gao N; Pearce DA; Lehrman MA; Hofmann SL. 2005. Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology 15(6):637-48. [PubMed: 15647513]  [MGI Ref ID J:112499]

Elshatory Y; Brooks AI; Chattopadhyay S; Curran TM; Gupta P; Ramalingam V; Hofmann SL; Pearce DA. 2003. Early changes in gene expression in two models of Batten disease. FEBS Lett 538(1-3):207-12. [PubMed: 12633880]  [MGI Ref ID J:119180]

Hu J; Lu JY; Wong AM; Hynan LS; Birnbaum SG; Yilmaz DS; Streit BM; Lenartowicz EM; Thompson TC; Cooper JD; Hofmann SL. 2012. Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis. Mol Genet Metab 107(1-2):213-21. [PubMed: 22704978]  [MGI Ref ID J:188057]

Khaibullina A; Kenyon N; Guptill V; Quezado MM; Wang L; Koziol D; Wesley R; Moya PR; Zhang Z; Saha A; Mukherjee AB; Quezado ZM. 2012. In a model of batten disease, palmitoyl protein thioesterase-1 deficiency is associated with brown adipose tissue and thermoregulation abnormalities. PLoS One 7(11):e48733. [PubMed: 23139814]  [MGI Ref ID J:194798]

Kielar C; Maddox L; Bible E; Pontikis CC; Macauley SL; Griffey MA; Wong M; Sands MS; Cooper JD. 2007. Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis 25(1):150-62. [PubMed: 17046272]  [MGI Ref ID J:134738]

Kielar C; Wishart TM; Palmer A; Dihanich S; Wong AM; Macauley SL; Chan CH; Sands MS; Pearce DA; Cooper JD; Gillingwater TH. 2009. Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease. Hum Mol Genet 18(21):4066-80. [PubMed: 19640925]  [MGI Ref ID J:153101]

Kim SJ; Zhang Z; Lee YC; Mukherjee AB. 2006. Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL. Hum Mol Genet 15(10):1580-6. [PubMed: 16571600]  [MGI Ref ID J:109538]

Kim SJ; Zhang Z; Sarkar C; Tsai PC; Lee YC; Dye L; Mukherjee AB. 2008. Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice. J Clin Invest 118(9):3075-86. [PubMed: 18704195]  [MGI Ref ID J:140851]

Macauley SL; Pekny M; Sands MS. 2011. The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis. J Neurosci 31(43):15575-85. [PubMed: 22031903]  [MGI Ref ID J:177265]

Palmer DN; Barry LA; Tyynela J; Cooper JD. 2013. NCL disease mechanisms. Biochim Biophys Acta 1832(11):1882-93. [PubMed: 23707513]  [MGI Ref ID J:202510]

Saha A; Kim SJ; Zhang Z; Lee YC; Sarkar C; Tsai PC; Mukherjee AB. 2008. RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis. FEBS Lett 582(27):3823-31. [PubMed: 18948101]  [MGI Ref ID J:140706]

Saha A; Sarkar C; Singh SP; Zhang Z; Munasinghe J; Peng S; Chandra G; Kong E; Mukherjee AB. 2012. The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol. Hum Mol Genet 21(10):2233-44. [PubMed: 22331300]  [MGI Ref ID J:183802]

Sarkar C; Chandra G; Peng S; Zhang Z; Liu A; Mukherjee AB. 2013. Neuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCL. Nat Neurosci 16(11):1608-17. [PubMed: 24056696]  [MGI Ref ID J:207427]

Tamaki SJ; Jacobs Y; Dohse M; Capela A; Cooper JD; Reitsma M; He D; Tushinski R; Belichenko PV; Salehi A; Mobley W; Gage FH; Huhn S; Tsukamoto AS; Weissman IL; Uchida N. 2009. Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis. Cell Stem Cell 5(3):310-9. [PubMed: 19733542]  [MGI Ref ID J:154123]

Virmani T; Gupta P; Liu X; Kavalali ET; Hofmann SL. 2005. Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice. Neurobiol Dis 20(2):314-23. [PubMed: 16242638]  [MGI Ref ID J:102821]

Wei H; Zhang Z; Saha A; Peng S; Chandra G; Quezado Z; Mukherjee AB. 2011. Disruption of adaptive energy metabolism and elevated ribosomal p-S6K1 levels contribute to INCL pathogenesis: partial rescue by resveratrol. Hum Mol Genet 20(6):1111-21. [PubMed: 21224254]  [MGI Ref ID J:168842]

Wendt KD; Lei B; Schachtman TR; Tullis GE; Ibe ME; Katz ML. 2005. Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze. Behav Brain Res 161(2):175-82. [PubMed: 15885820]  [MGI Ref ID J:98844]

Zhang Z; Lee YC; Kim SJ; Choi MS; Tsai PC; Saha A; Wei H; Xu Y; Xiao YJ; Zhang P; Heffer A; Mukherjee AB. 2007. Production of lysophosphatidylcholine by cPLA2 in the brain of mice lacking PPT1 is a signal for phagocyte infiltration. Hum Mol Genet 16(7):837-47. [PubMed: 17341491]  [MGI Ref ID J:121809]

Zhang Z; Lee YC; Kim SJ; Choi MS; Tsai PC; Xu Y; Xiao YJ; Zhang P; Heffer A; Mukherjee AB. 2006. Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL. Hum Mol Genet 15(2):337-46. [PubMed: 16368712]  [MGI Ref ID J:104521]

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 originated on a 129S6 background. Heterozygotes were bred to C57BL/6 mice and then intercrossed to produce homozygotes. The strain is maintained on a B6;129S6 background.

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 willfulfill 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 willfulfill 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.

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

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.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


(6.6)