Strain Name:

B6;129P2-Pnpla2tm1Rze/J

Stock Number:

019003

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Availability:

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In this ATGL-ko strain a NEO cassette replaces exon 1 of the Pnpla2 (patatin-like phospholipase domain containing 2) gene. Homozygotes exhibit a shortened lifespan, abnormal lipid homeostasis, glucose tolerance, and cardiovascular physiology and have applications in studies of impaired cardiac function, thermogenesis and lipid and energy homeostasis.

Description

Strain Information

Former Names B6.129P2-Pnpla2tm1Rze/J    (Changed: 03-SEP-13 )
Type Mutant Stock; Targeted Mutation;
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Mating SystemWild-type x Heterozygote         (Female x Male)   13-AUG-14
Mating SystemHeterozygote x Wild-type         (Female x Male)   13-AUG-14
Specieslaboratory mouse
Generation+PN1F0 (27-DEC-12)
Generation Definitions
 
Donating Investigator Rudolf Zechner,   Karl Franzens University Graz

Description
Patatin-like phospholipase domain containing 2 (PNPLA2, ATGL) initiates lipolysis by catalyzing the hydrolysis of triacylglycerol to diacylglycerol in adipose tissue and non-adipose tissues. Mutations in human PNPLA2 have been associated with neutral lipids storage disorder with myopathy. These mice carry a targeted mutation of the Pnpla2 gene in which exon 1 (encoding the translational initiation codon and the lipase consensus sequence motif,) is replaced by a NEO cassette. Mice that are heterozygous for the targeted mutation are viable and fertile. Homozygotes die at about 14 to 16 weeks of age due to severe cardiac steatosis and lethal myopathy. Triacylglycerol hydrolase activity in homozygotes is decreased by more than 80% in white and brown adipose tissue, and reduced in cardiac muscle, skeletal muscle, testis, and in the liver. Levels of plasma free fatty acids are reduced in both fed and fasting homozygotes. Mice homozygous for this targeted mutation have 2 fold increased body mass, as well as increased gonadal and inguinal white adipose tissue. Intrascapular brown adipose tissue is increased more than 10-fold when compared to wildtype controls. Homozygotes exhibit increased levels of triacylglycerol also in most non-adipose tissues with a more than 10-fold increase in cardiac muscle. Following an overnight fast, homozygotes are hypoinsulinemic (ciruculating insulin levels reduced by 70%) and hypoglycemic (reduced approximately 40%). Fed homozygous mice exhibit a 40% reduction in circulating insulin compared to levels seen in controls. Homozygotes exhibit increased glucose tolerance and increased respiratory quotient during fasting. Homozygotes are sensitive to cold temperatures, experiencing severe hypothermia after 5 hours in 4 degrees celcius. Homozygous are protected against certain forms of cancer cachexia.

Development
A targeting vector containing a NEO cassette was used to disrupt exon 1, which encodes the translational initiation codon and the lipase consensus sequence motif (GXSXG). The construct was electroporated into 129P2/OlaHsd-Hprtb-m3 derived HM-1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting chimeric animals were crossed to C57BL/6J mice. The donating investigator reported that these mice were backcrossed to C57BL/6J for at least 10 generations (see SNP note below). Upon arrival at The Jackson Laboratory, the mice were crossed to C57BL/6J (Stock No. 000664) at least once to establish the colony.

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. Three markers throughout the genome were segregating for 129, suggesting an incomplete backcross.

Control Information

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

Related Strains

Strains carrying other alleles of Pnpla2
024278   B6N.129S-Pnpla2tm1Eek/J
024277   FVB-Tg(Ckm-Pnpla2)164Eek/J
View Strains carrying other alleles of Pnpla2     (2 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.
Neutral Lipid Storage Disease with Myopathy; NLSDM   (PNPLA2)
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.

Pnpla2tm1Rze/Pnpla2tm1Rze

        involves: 129P2/OlaHsd * C57BL/6
  • mortality/aging
  • premature death
    • pronounced cardiac dysfunction results in premature death for male and female homozygotes; the first mice die around 12 weeks of age   (MGI Ref ID J:108546)
    • male mice (50% after 16 weeks) die earlier than female (50% after 20 weeks)   (MGI Ref ID J:108546)
    • heterozygous mice have normal life spans   (MGI Ref ID J:108546)
  • growth/size/body phenotype
  • increased body mass index
    • homozygotes display a 2-fold increase in body fat mass   (MGI Ref ID J:108546)
  • increased body weight
    • homozygotes are heavier than wild-type   (MGI Ref ID J:108546)
  • homeostasis/metabolism phenotype
  • abnormal homeostasis   (MGI Ref ID J:108546)
    • abnormal circulating glucose level   (MGI Ref ID J:108546)
      • decreased circulating glucose level
        • after a 6-hour fast, mice have significantly lower basal glucose values compared to wild-type   (MGI Ref ID J:108546)
    • abnormal circulating hormone level   (MGI Ref ID J:108546)
      • abnormal circulating insulin level   (MGI Ref ID J:108546)
        • decreased circulating insulin level
          • in the fed state, plasma insulin levels are reduced by 42% in mutants compared to wild-type   (MGI Ref ID J:108546)
      • increased circulating leptin level
        • plasma leptin levels are elevated by 2.1-fold in fed mutants and by 4.4-fold in fasted mutant mice   (MGI Ref ID J:108546)
    • abnormal gas homeostasis   (MGI Ref ID J:108546)
      • abnormal respiratory quotient
        • RQ in mutants deviates from wild-type during fasting; RQ remains constant whereas in wild-type, it decreases with increasing fasting time   (MGI Ref ID J:108546)
      • decreased oxygen consumption
        • oxygen consumption decreases to 25% of that of wild-type after 18 hours of fasting   (MGI Ref ID J:108546)
    • abnormal glucose tolerance   (MGI Ref ID J:108546)
      • improved glucose tolerance
        • after a 6-hour fast, null mice show improved glucose tolerance compared to wild-type   (MGI Ref ID J:108546)
    • abnormal lipid homeostasis   (MGI Ref ID J:108546)
      • decreased circulating HDL cholesterol level
        • total cholesterol (due to decreased HDL levels ) is reduced by 22%   (MGI Ref ID J:108546)
      • decreased circulating free fatty acid level
        • in mutants, levels are reduced by 30% in the fed state and by 62% in the fasted state   (MGI Ref ID J:108546)
      • decreased circulating triglyceride level
        • levels are decreased by 66%   (MGI Ref ID J:108546)
        • decreased circulating VLDL triglyceride level
          • reduced VLDL levels are found in mutants   (MGI Ref ID J:108546)
      • impaired lipolysis
        • in mutants there is decreased lipolytic activities in white (-82%) and brown (-85%) adipose tissues, cardiac muscle (-31%), skeletal muscle (-44%), testis (-69%) and liver (-73%)   (MGI Ref ID J:108546)
      • increased triglyceride level
        • there is a 20-fold increase in triglyceride content in cardiac muscle by 12 weeks of age in mutants   (MGI Ref ID J:108546)
        • in non-cardiac tissue the most pronounced accumulation of triglycerides is a >10-fold increase seen in the testis and kidney; smaller (1.5-4-fold) increases are seen in all tissues including the liver   (MGI Ref ID J:108546)
    • cardiac edema
    • impaired adaptive thermogenesis
      • after 5 hours of cold exposure, mutant body temperature is only 25 degrees C   (MGI Ref ID J:108546)
      • when mutants are exposed to cold for 5 hours, they suffer from life-threatening hypothermia as a result of impaired catabolism to triglycerides to brown adipose tissue   (MGI Ref ID J:108546)
      • after 18 hours of fasting, body temperature drops to 28.4 degrees C vs 35.4 degrees in wild-type with prolonged fasting   (MGI Ref ID J:108546)
    • increased insulin sensitivity
      • maximal decline of blood glucose levels is more pronounced (34 mg/dl vs 53 mg/dl in wild-type) in Pnpla-deficient mice and persists for the entire 3 hour observation period   (MGI Ref ID J:108546)
  • adipose tissue phenotype
  • abnormal brown adipose tissue morphology
    • mutant mice have enlarged lipid droplets (1395 micrometer vs 67 micrometer in wild-type) in brown adipocytes   (MGI Ref ID J:108546)
    • increased brown adipose tissue amount
      • mutants have an 8.5 fold increase in wet weight of intracapsular brown adipose tissue   (MGI Ref ID J:108546)
  • abnormal white adipose tissue morphology
    • mutant mice have enlarged lipid droplets (4690 micrometer vs 3382 micrometer in wild-type) in white adipocytes   (MGI Ref ID J:108546)
  • increased gonadal fat pad weight
    • homozygotes display a 1.6 fold increase in wet weight of gonadal white adipose tissue compared to controls   (MGI Ref ID J:108546)
  • increased inguinal fat pad weight
    • homozygotes display a 2.1 fold increase in wet weight of inguinal white adipose tissue compared to controls   (MGI Ref ID J:108546)
  • increased interscapular fat pad weight
    • mutants have an 8.5 fold increase in wet weight of intracapsular brown adipose tissue   (MGI Ref ID J:108546)
  • cardiovascular system phenotype
  • abnormal cardiovascular system physiology
    • massive lipid buildup causes severe cardiac insufficiency in mutants   (MGI Ref ID J:108546)
    • at death, cardiac edema and pleural as well as cardiac effusions are observed; these are not observed in mice before 14 weeks of age   (MGI Ref ID J:108546)
    • abnormal cardiomyocyte apoptosis
      • in mutants there is a moderate induction of apoptosis   (MGI Ref ID J:108546)
    • congestive heart failure
      • at death, mutants display typical features of congestive heart failure   (MGI Ref ID J:108546)
    • decreased ventricle muscle contractility
      • ejection fraction from left ventricle is drastically reduced compared to wild-type (40.2% vs 80.5% in wild-type)   (MGI Ref ID J:108546)
  • abnormal heart morphology   (MGI Ref ID J:108546)
    • abnormal myocardium layer morphology
      • in homozygotes there is severe triglyceride accumulation in cardiac muscle; at 12 weeks of age, mutants have a 20-fold higher triglyceride content in myocytes compared to wild-type   (MGI Ref ID J:108546)
      • abnormal myocardial fiber morphology
        • lipid droplets show an age-dependent increase in size and number in cardiomyocytes of mutants beginning with microvesicular droplets at 6 weeks and progressing to large droplets by 18 weeks   (MGI Ref ID J:108546)
    • cardiac fibrosis
      • there is a marked change in cardiac texture and increase in fibrosis   (MGI Ref ID J:108546)
    • dilated heart left ventricle
      • at death; this is not observed in mice before 14 weeks of age   (MGI Ref ID J:108546)
    • dilated heart right ventricle
      • at death; this is not observed in mice before 14 weeks of age   (MGI Ref ID J:108546)
    • increased heart weight
      • mutant hearts have a 1.4x heart weight because of the excess triglycerides (193 mg for mutant vs 131 mg wild-type)   (MGI Ref ID J:108546)
    • thick interventricular septum
      • the interventricular septum in mutant hearts is significantly thickened and increases in thickness with age   (MGI Ref ID J:108546)
  • pulmonary vascular congestion
    • congestion is observed in pulmonary vessels at death; not observed in mice before 14 weeks of age   (MGI Ref ID J:108546)
  • muscle phenotype
  • abnormal cardiomyocyte apoptosis
    • in mutants there is a moderate induction of apoptosis   (MGI Ref ID J:108546)
  • abnormal myocardial fiber morphology
    • lipid droplets show an age-dependent increase in size and number in cardiomyocytes of mutants beginning with microvesicular droplets at 6 weeks and progressing to large droplets by 18 weeks   (MGI Ref ID J:108546)
  • decreased ventricle muscle contractility
    • ejection fraction from left ventricle is drastically reduced compared to wild-type (40.2% vs 80.5% in wild-type)   (MGI Ref ID J:108546)
  • liver/biliary system phenotype
  • hepatic steatosis   (MGI Ref ID J:108546)
  • respiratory system phenotype
  • pulmonary vascular congestion
    • congestion is observed in pulmonary vessels at death; not observed in mice before 14 weeks of age   (MGI Ref ID J:108546)
  • cellular phenotype
  • abnormal cardiomyocyte apoptosis
    • in mutants there is a moderate induction of apoptosis   (MGI Ref ID J:108546)

Pnpla2tm1Rze/Pnpla2tm1Rze

        involves: 129P2/OlaHsd
  • mortality/aging
  • premature death
    • however, treatment with Wy14643 exhibit increased survival   (MGI Ref ID J:176252)
  • cardiovascular system phenotype
  • abnormal cardiovascular system morphology
    • treatment with GW501516 or GW0742 (PPARG agonists) fail to improve cardiometabolic defects   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) rescues cardiometabolic defects   (MGI Ref ID J:176252)
    • abnormal heart ventricle morphology
      • thickened posterior wall   (MGI Ref ID J:176252)
      • lower left ventricular end-diastolic dimensions   (MGI Ref ID J:176252)
      • increased left ventricle weight
        • however, treatment with Wy14643 (PPARA agonist) restores normal left ventricle weight   (MGI Ref ID J:176252)
      • thick interventricular septum
        • however, treatment with Wy14643 (PPARA agonist) reduces interventricular septum thickness   (MGI Ref ID J:176252)
    • abnormal myocardial fiber morphology
      • cardiomyocytes exhibit increased size and number of lipid droplets and glycogen granules compared to in wild-type muscles   (MGI Ref ID J:176252)
      • cardiomyocytes exhibit increased mitochondria size with decreased mitochondrial DNA compared to in wild-type muscles   (MGI Ref ID J:176252)
      • however, treatment with Wy14643 (PPARA agonist) restores mitochondrial size and membrane potential   (MGI Ref ID J:176252)
    • cardiac hypertrophy
      • concentric hypertrophy   (MGI Ref ID J:176252)
  • abnormal myocardial fiber physiology
    • cardiomyocytes exhibit reduced oxidative capacity compared with wild-type mice   (MGI Ref ID J:176252)
  • decreased ventricle muscle contractility
    • mice exhibit reduced ventricular fractional shortening and ejection fraction compared with wild-type mice   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) restores fractional shortening and ejection fraction   (MGI Ref ID J:176252)
  • increased heart rate
    • however, treatment with Wy14643 (PPARA agonist) reduces heart rate   (MGI Ref ID J:176252)
  • homeostasis/metabolism phenotype
  • abnormal lipid homeostasis
    • mouse embryonic fibroblasts treated with oleic acid to induce lipogenesis exhibit a 1.9-fold increase in triglyceride accumulation compared with similarly treated wild-type cells   (MGI Ref ID J:160725)
    • decreased circulating free fatty acid level   (MGI Ref ID J:176252)
    • decreased circulating triglyceride level   (MGI Ref ID J:176252)
    • impaired lipolysis
      • mouse embryonic fibroblasts exhibit decreased triglyceride hydrolase activity compared with wild-type cells   (MGI Ref ID J:160725)
      • liver and skin triglyceride hydrolase activity is decreased compared to in wild-type mice   (MGI Ref ID J:160725)
      • however, epidermal triglyceride hydrolase activity is normal   (MGI Ref ID J:160725)
    • increased fatty acid level
      • mice exhibit a 53% increase in total intracellular concentration of unesterified fatty acid in the heart compared with wild-type mice   (MGI Ref ID J:176252)
    • increased triglyceride level
      • 4.6-fold in the livers of newborn mice   (MGI Ref ID J:160725)
      • 1.6-fold in the dermal   (MGI Ref ID J:160725)
      • 1.3-fold in epidermis   (MGI Ref ID J:160725)
      • in cardiac and liver muscles   (MGI Ref ID J:176252)
      • 4.6-fold higher in older mice than in younger mice   (MGI Ref ID J:176252)
      • however, treatment with Wy14643 or fenofibrate (PPARA agonists) restores normal triglyceride levels   (MGI Ref ID J:176252)
      • increased liver triglyceride level
        • 4.6-fold in the livers of newborn mice   (MGI Ref ID J:160725)
        • however, treatment with Wy14643 or fenofibrate (PPARA agonists) reduces triglyceride levels   (MGI Ref ID J:176252)
  • abnormal metabolism
    • treatment with GW501516 or GW0742 (PPARG agonists) fail to improve cardiometabolic defects   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) rescues cardiometabolic defects   (MGI Ref ID J:176252)
    • abnormal enzyme/coenzyme activity
      • mouse embryonic fibroblasts exhibit decreased triglyceride hydrolase activity compared with wild-type cells   (MGI Ref ID J:160725)
      • liver and skin triglyceride hydrolase activity is decreased compared to in wild-type mice   (MGI Ref ID J:160725)
      • however, epidermal triglyceride hydrolase activity is normal   (MGI Ref ID J:160725)
  • abnormal respiratory quotient
    • during the light period or food deprivation, mice exhibit less of a decrease in respiratory quotient compared with wild-type mice   (MGI Ref ID J:176252)
    • increased respiratory quotient   (MGI Ref ID J:176252)
  • decreased oxygen consumption
    • in heart homogenate mitochondria under basal and succinate-stimulated conditions   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) restores oxygen consumptions   (MGI Ref ID J:176252)
  • increased glycogen level
    • in cardiac muscles   (MGI Ref ID J:176252)
  • adipose tissue phenotype
  • increased brown adipose tissue amount   (MGI Ref ID J:176252)
  • increased white adipose tissue amount   (MGI Ref ID J:176252)
  • cellular phenotype
  • abnormal mitochondrion morphology
    • cardiomyocytes exhibit increased mitochondria size with decreased mitochondrial DNA compared to in wild-type muscles   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) restores mitochondrial size and membrane potential   (MGI Ref ID J:176252)
  • liver/biliary system phenotype
  • increased liver triglyceride level
    • 4.6-fold in the livers of newborn mice   (MGI Ref ID J:160725)
    • however, treatment with Wy14643 or fenofibrate (PPARA agonists) reduces triglyceride levels   (MGI Ref ID J:176252)
  • integument phenotype
  • *normal* integument phenotype
    • mice exhibit normal skin barrier function   (MGI Ref ID J:160725)
  • muscle phenotype
  • abnormal myocardial fiber morphology
    • cardiomyocytes exhibit increased size and number of lipid droplets and glycogen granules compared to in wild-type muscles   (MGI Ref ID J:176252)
    • cardiomyocytes exhibit increased mitochondria size with decreased mitochondrial DNA compared to in wild-type muscles   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) restores mitochondrial size and membrane potential   (MGI Ref ID J:176252)
  • decreased ventricle muscle contractility
    • mice exhibit reduced ventricular fractional shortening and ejection fraction compared with wild-type mice   (MGI Ref ID J:176252)
    • however, treatment with Wy14643 (PPARA agonist) restores fractional shortening and ejection fraction   (MGI Ref ID J:176252)
View Research Applications

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

Cardiovascular Research
Other
      altered fat metabolism

Developmental Biology Research
Postnatal Lethality
      Homozygous

Diabetes and Obesity Research
Hypoglycemia
Hypoinsulinemia

Internal/Organ Research
Adipose Defects

Metabolism Research
Enzyme Deficiency
Lipid Metabolism

Neurobiology Research
Metabolic Defects

Research Tools
Diabetes and Obesity Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Pnpla2tm1Rze
Allele Name targeted mutation 1, Rudolf Zechner
Allele Type Targeted (Null/Knockout)
Common Name(s) ATGL-ko; ATGL-;
Mutation Made By Rudolf Zechner,   Karl Franzens University Graz
Strain of Origin129P2/OlaHsd-Hprt
Gene Symbol and Name Pnpla2, patatin-like phospholipase domain containing 2
Chromosome 7
Gene Common Name(s) 0610039C21Rik; 1110001C14Rik; ATGL; PEDF-R; RGD1309044; RIKEN cDNA 0610039C21 gene; RIKEN cDNA 1110001C14 gene; TTS-2.2; TTS2; desnutrin; iPLA2zeta;
Molecular Note A neomycin resistance cassette replaced the first exon including the translational start codon and the lipase consensus sequence motif (GXSXG). Northern analysis of white adipose tissue did not detect transcript in white adipose tissue from homozygous mutant mice. Similarly, western blot analysis of white adipose tissue from homozygous mutant mice did not detect protein. [MGI Ref ID J:108546]

Genotyping

Genotyping Information

Genotyping Protocols

Pnpla2tm1Rze,

Separated MCA


Pnpla2tm1Rze, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Kaneko T; Maeda A; Takefuji M; Aoyama H; Nakayama M; Kawabata S; Kawano Y; Iwamatsu A; Amano M; Kaibuchi K. 2005. Rho mediates endocytosis of epidermal growth factor receptor through phosphorylation of endophilin A1 by Rho-kinase. Genes Cells 10(10):973-87. [PubMed: 16164598]  [MGI Ref ID J:108456]

Additional References

Pnpla2tm1Rze related

Aflaki E; Radovic B; Chandak PG; Kolb D; Eisenberg T; Ring J; Fertschai I; Uellen A; Wolinski H; Kohlwein SD; Zechner R; Levak-Frank S; Sattler W; Graier WF; Malli R; Madeo F; Kratky D. 2011. Triacylglycerol accumulation activates the mitochondrial apoptosis pathway in macrophages. J Biol Chem 286(9):7418-28. [PubMed: 21196579]  [MGI Ref ID J:170551]

Borg ML; Andrews ZB; Duh EJ; Zechner R; Meikle PJ; Watt MJ. 2011. Pigment Epithelium-Derived Factor Regulates Lipid Metabolism via Adipose Triglyceride Lipase. Diabetes 60(5):1458-66. [PubMed: 21464445]  [MGI Ref ID J:171814]

Das SK; Eder S; Schauer S; Diwoky C; Temmel H; Guertl B; Gorkiewicz G; Tamilarasan KP; Kumari P; Trauner M; Zimmermann R; Vesely P; Haemmerle G; Zechner R; Hoefler G. 2011. Adipose triglyceride lipase contributes to cancer-associated cachexia. Science 333(6039):233-8. [PubMed: 21680814]  [MGI Ref ID J:173533]

Eichmann TO; Kumari M; Haas JT; Farese RV Jr; Zimmermann R; Lass A; Zechner R. 2012. Studies on the substrate and stereo/regioselectivity of adipose triglyceride lipase, hormone-sensitive lipase, and diacylglycerol-O-acyltransferases. J Biol Chem 287(49):41446-57. [PubMed: 23066022]  [MGI Ref ID J:192630]

Etschmaier K; Becker T; Eichmann TO; Schweinzer C; Scholler M; Tam-Amersdorfer C; Poeckl M; Schuligoi R; Kober A; Chirackal Manavalan AP; Rechberger GN; Streith IE; Zechner R; Zimmermann R; Panzenboeck U. 2011. Adipose triglyceride lipase affects triacylglycerol metabolism at brain barriers. J Neurochem 119(5):1016-28. [PubMed: 21951135]  [MGI Ref ID J:178474]

Haemmerle G; Lass A; Zimmermann R; Gorkiewicz G; Meyer C; Rozman J; Heldmaier G; Maier R; Theussl C; Eder S; Kratky D; Wagner EF; Klingenspor M; Hoefler G; Zechner R. 2006. Defective lipolysis and altered energy metabolism in mice lacking adipose triglyceride lipase. Science 312(5774):734-7. [PubMed: 16675698]  [MGI Ref ID J:108546]

Haemmerle G; Moustafa T; Woelkart G; Buttner S; Schmidt A; van de Weijer T; Hesselink M; Jaeger D; Kienesberger PC; Zierler K; Schreiber R; Eichmann T; Kolb D; Kotzbeck P; Schweiger M; Kumari M; Eder S; Schoiswohl G; Wongsiriroj N; Pollak NM; Radner FP; Preiss-Landl K; Kolbe T; Rulicke T; Pieske B; Trauner M; Lass A; Zimmermann R; Hoefler G; Cinti S; Kershaw EE; Schrauwen P; Madeo F; Mayer B; Zechner R. 2011. ATGL-mediated fat catabolism regulates cardiac mitochondrial function via PPAR-alpha and PGC-1. Nat Med 17(9):1076-85. [PubMed: 21857651]  [MGI Ref ID J:176252]

Huijsman E; van de Par C; Economou C; van der Poel C; Lynch GS; Schoiswohl G; Haemmerle G; Zechner R; Watt MJ. 2009. Adipose triacylglycerol lipase deletion alters whole body energy metabolism and impairs exercise performance in mice. Am J Physiol Endocrinol Metab 297(2):E505-13. [PubMed: 19491295]  [MGI Ref ID J:151431]

Kienesberger PC; Lee D; Pulinilkunnil T; Brenner DS; Cai L; Magnes C; Koefeler HC; Streith IE; Rechberger GN; Haemmerle G; Flier JS; Zechner R; Kim YB; Kershaw EE. 2009. Adipose triglyceride lipase deficiency causes tissue-specific changes in insulin signaling. J Biol Chem 284(44):30218-29. [PubMed: 19723629]  [MGI Ref ID J:155840]

Lin Y; Chiba S; Suzuki A; Yamaguchi S; Nakanishi T; Matsumoto H; Ikeda Y; Ishibashi-Ueda H; Hirano K; Kato S. 2013. Vascular smooth muscle cells isolated from adipose triglyceride lipase-deficient mice exhibit distinct phenotype and phenotypic plasticity. Biochem Biophys Res Commun 434(3):534-40. [PubMed: 23583398]  [MGI Ref ID J:201857]

Pagnon J; Matzaris M; Stark R; Meex RC; Macaulay SL; Brown W; O'Brien PE; Tiganis T; Watt MJ. 2012. Identification and functional characterization of protein kinase A phosphorylation sites in the major lipolytic protein, adipose triglyceride lipase. Endocrinology 153(9):4278-89. [PubMed: 22733971]  [MGI Ref ID J:189193]

Peyot ML; Guay C; Latour MG; Lamontagne J; Lussier R; Pineda M; Ruderman NB; Haemmerle G; Zechner R; Joly E; Madiraju SR; Poitout V; Prentki M. 2009. Adipose Triglyceride Lipase Is Implicated in Fuel- and Non-fuel-stimulated Insulin Secretion. J Biol Chem 284(25):16848-59. [PubMed: 19389712]  [MGI Ref ID J:151207]

Pinent M; Hackl H; Burkard TR; Prokesch A; Papak C; Scheideler M; Hammerle G; Zechner R; Trajanoski Z; Strauss JG. 2008. Differential transcriptional modulation of biological processes in adipocyte triglyceride lipase and hormone-sensitive lipase-deficient mice. Genomics 92(1):26-32. [PubMed: 18572100]  [MGI Ref ID J:137455]

Pulinilkunnil T; Kienesberger PC; Nagendran J; Waller TJ; Young ME; Kershaw EE; Korbutt G; Haemmerle G; Zechner R; Dyck JR. 2013. Myocardial adipose triglyceride lipase overexpression protects diabetic mice from the development of lipotoxic cardiomyopathy. Diabetes 62(5):1464-77. [PubMed: 23349479]  [MGI Ref ID J:208574]

Radner FP; Streith IE; Schoiswohl G; Schweiger M; Kumari M; Eichmann TO; Rechberger G; Koefeler HC; Eder S; Schauer S; Theussl HC; Preiss-Landl K; Lass A; Zimmermann R; Hoefler G; Zechner R; Haemmerle G. 2010. Growth retardation, impaired triacylglycerol catabolism, hepatic steatosis, and lethal skin barrier defect in mice lacking comparative gene identification-58 (CGI-58). J Biol Chem 285(10):7300-11. [PubMed: 20023287]  [MGI Ref ID J:160725]

Schoiswohl G; Schweiger M; Schreiber R; Gorkiewicz G; Preiss-Landl K; Taschler U; Zierler KA; Radner FP; Eichmann TO; Kienesberger PC; Eder S; Lass A; Haemmerle G; Alsted TJ; Kiens B; Hoefler G; Zechner R; Zimmermann R. 2010. Adipose triglyceride lipase plays a key role in the supply of the working muscle with fatty acids. J Lipid Res 51(3):490-9. [PubMed: 19965578]  [MGI Ref ID J:159033]

Schrammel A; Mussbacher M; Winkler S; Haemmerle G; Stessel H; Wolkart G; Zechner R; Mayer B. 2013. Cardiac oxidative stress in a mouse model of neutral lipid storage disease. Biochim Biophys Acta 1831(11):1600-8. [PubMed: 23867907]  [MGI Ref ID J:204107]

Shimizu I; Yoshida Y; Katsuno T; Tateno K; Okada S; Moriya J; Yokoyama M; Nojima A; Ito T; Zechner R; Komuro I; Kobayashi Y; Minamino T. 2012. p53-induced adipose tissue inflammation is critically involved in the development of insulin resistance in heart failure. Cell Metab 15(1):51-64. [PubMed: 22225876]  [MGI Ref ID J:182322]

Sitnick MT; Basantani MK; Cai L; Schoiswohl G; Yazbeck CF; Distefano G; Ritov V; DeLany JP; Schreiber R; Stolz DB; Gardner NP; Kienesberger PC; Pulinilkunnil T; Zechner R; Goodpaster BH; Coen P; Kershaw EE. 2013. Skeletal muscle triacylglycerol hydrolysis does not influence metabolic complications of obesity. Diabetes 62(10):3350-61. [PubMed: 23835334]  [MGI Ref ID J:207007]

Turpin SM; Hoy AJ; Brown RD; Garcia Rudaz C; Honeyman J; Matzaris M; Watt MJ. 2010. Adipose triacylglycerol lipase is a major regulator of hepatic lipid metabolism but not insulin sensitivity in mice. Diabetologia :. [PubMed: 20842343]  [MGI Ref ID J:164672]

van de Weijer T; van Ewijk PA; Zandbergen HR; Slenter JM; Kessels AG; Wildberger JE; Hesselink MK; Schrauwen P; Schrauwen-Hinderling VB; Kooi ME. 2012. Geometrical models for cardiac MRI in rodents: comparison of quantification of left ventricular volumes and function by various geometrical models with a full-volume MRI data set in rodents. Am J Physiol Heart Circ Physiol 302(3):H709-15. [PubMed: 22101529]  [MGI Ref ID J:182364]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX18

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice can be bred as heterozygotes. Homozygotes die at about 14 to 16 weeks of age.
Mating SystemWild-type x Heterozygote         (Female x Male)   13-AUG-14
Heterozygote x Wild-type         (Female x Male)   13-AUG-14
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 $199.90Female or MaleHeterozygous for Pnpla2tm1Rze  
Price per Pair (US dollars $)Pair Genotype
$271.90Heterozygous for Pnpla2tm1Rze x Wild-type for Pnpla2tm1Rze  
$271.90Wild-type for Pnpla2tm1Rze x Heterozygous for Pnpla2tm1Rze  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $259.90Female or MaleHeterozygous for Pnpla2tm1Rze  
Price per Pair (US dollars $)Pair Genotype
$353.50Heterozygous for Pnpla2tm1Rze x Wild-type for Pnpla2tm1Rze  
$353.50Wild-type for Pnpla2tm1Rze x Heterozygous for Pnpla2tm1Rze  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Control Information

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

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.


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