Strain Name:

B6.129-Ppargc1atm1Brsp/J

Stock Number:

008597

Order this mouse

Availability:

Repository- Live

Mice that are homozygous for this Ppargc1a (peroxisome proliferative activated receptor, gamma, coactivator 1 alpha) knock-out mutation exhibit some postnatal lethality, impaired mitochondrial function and gluconeogenesis, are hypermetabolic, hyperactive, and sensitive to cold temperatures. This mutant mouse strain may be useful in studies of metabolic homeostasis, resistance to obesity, hyperactivity and behavior, mitochondrial impairment and neurodegeneration, and heart failure.

Description

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.
Mating System+/+ sibling x Heterozygote         (Female x Male)   08-AUG-09
Specieslaboratory mouse
GenerationN10+F11 (12-DEC-13)
Generation Definitions
 
Donating Investigator Bruce Spiegelman,   Dana-Farber Cancer Institute

Description
Mice that are homozygous for this targeted mutation are fertile, normal in size and do not display any gross physical or behavioral abnormalities. Approximately half of homozygotes exhibit postnatal lethality. The Donating Investigator reports maintaining homozygous pups at a higher temperature (77°F) increases their survival. No gene product (mRNA or protein) is detected by RNA hybridization, real-time PCR analysis of skeletal muscle or liver, or Western blot analysis of brown fat. Histological examination of the brown fat from homozygotes reveals abnormal accumulation of large lipid droplets. Examination of brain tissue shows spongiform lesions and gliosis. When fed a high fat diet homozygotes have increased insulin sensitivity, glucose tolerance and reduced body weight. After 24 hours of fasting, homozygotes develop mild hypoglycemia. Mutants have impaired mitochondrial function and gluconeogenesis and are hypermetabolic as well as hyperactive. Homozygotes are unable to survive exposure to 4°C for more than 6 hours. Exaggerated startle response, stimulus induced myoclonus, dystonic posturing, and limb clasping are also observed. Transverse aortic constriction (TAC) results in dilated cardiomyopathy and development of heart failure. The Donating Investigator reports that heterozygote X heterozygote crosses yields less than the expected Mendelian ratio (the observed ratio is 1 homozygote in 8); and homozygous mice are more likely to have litters disappear or die after birth. This mutant mouse strain may be useful in studies of metabolic homeostasis, resistance to obesity, hyperactivity and behavior, mitochondrial impairment, neurodegeneration, and heart failure.

Development
A loxP site flanked targeting vector containing hygromycin resistance and thymidine kinase genes utilized in the construction of this mutant. This selection cassette was inserted downstream of exon 5 of the targeted gene, and another loxP site was inserted upstream of exon 3. This construct was electroporated into 129 derived embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. The resulting chimeric animals were crossed to C57BL/6 mice. The resulting offspring were then crossed to transgenic mice (on the C57BL/6 genetic background) expressing Cre recombinase under the control of the zona pellucida 3 (Zp3) promoter to remove exons 3 through 5 and the selection cassette. Heterozygotes were crossed to generate homozygotes. The donating investigator reported that the mice were then backcrossed to C57BL/6 (see SNP note below) for 9 generations before arriving at The Jackson Laboratory.

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

Control Information

  Control
   Wild-type from the colony
   101043 B6129SF1/J (approximate)
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Ppargc1a
009666   B6.129-Ppargc1atm2.1Brsp/J
009662   B6.129X1-Ppargc1atm1Dpk/J
008231   C57BL/6-Tg(Ckm-Ppargc1a)31Brsp/J
012387   FVB-Tg(tetO-Ppargc1a)1Dpk/J
View Strains carrying other alleles of Ppargc1a     (4 strains)

Phenotype

Phenotype Information

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.

Ppargc1atm1Brsp/Ppargc1a+

        involves: 129
  • immune system phenotype
  • *normal* immune system phenotype
    • mice exhibit normal serum IL6 levels   (MGI Ref ID J:127403)

Ppargc1atm1Brsp/Ppargc1atm1Brsp

        involves: 129/Sv * C57BL/6
  • mortality/aging
  • increased sensitivity to induced morbidity/mortality
    • homozygotes die when exposed to 4oC for more than 6 hours   (MGI Ref ID J:93896)
  • partial postnatal lethality
    • about 50% of homozygotes die during the postnatal period   (MGI Ref ID J:93896)
  • adipose tissue phenotype
  • abnormal brown adipose tissue morphology
    • abundant, large lipid droplets are seen in the brown fat   (MGI Ref ID J:93896)
  • behavior/neurological phenotype
  • dystonia
    • dystonic posturing is seen   (MGI Ref ID J:93896)
  • hyperactivity
    • a 40% increase in the frequency of random movements is seen   (MGI Ref ID J:93896)
  • increased startle reflex
    • an exaggerated startle response is seen   (MGI Ref ID J:93896)
  • limb grasping   (MGI Ref ID J:93896)
  • myoclonus
    • stimulus induced myoclonus is seen   (MGI Ref ID J:93896)
  • cellular phenotype
  • abnormal cellular respiration
    • respiration due to mitochondrial proton leak is increased 20% in primary hepatocytes   (MGI Ref ID J:93896)
  • homeostasis/metabolism phenotype
  • abnormal glucose homeostasis   (MGI Ref ID J:93896)
    • abnormal gluconeogenesis
      • conversion of pyruvate into glucose is reduced following intraperitoneal pyruvate injection   (MGI Ref ID J:93896)
    • decreased circulating insulin level
      • fed but not fasted mutants have decreased insulin levels relative to fed or fasted wild-type mice, respectively   (MGI Ref ID J:93896)
    • hypoglycemia
      • fasted mutants develop mild hypoglycemia after 24 hours   (MGI Ref ID J:93896)
    • improved glucose tolerance
      • on a high fat diet homozygous mutants have significantly increased glucose tolerance compared to wild-type mice   (MGI Ref ID J:93896)
    • increased insulin sensitivity
      • on a high fat diet homozygous mutants have significantly increased insulin sensitivity compared to wild-type mice   (MGI Ref ID J:93896)
  • abnormal lipid homeostasis   (MGI Ref ID J:93896)
    • decreased liver triglyceride level
      • triglyceride levels are lower in the livers of fasted mutant mice   (MGI Ref ID J:93896)
  • decreased susceptibility to diet-induced obesity
    • on a high fat diet homozygous mutants are significantly leaner compared to wild-type mice   (MGI Ref ID J:93896)
  • impaired adaptive thermogenesis
    • mutants are unable to maintain body temperature in response to cold temperatures developing lethal hypothermia when exposed to 4oC for more than 6 hours   (MGI Ref ID J:93896)
  • increased oxygen consumption
    • total oxygen consumption is increased 17% in primary hepaotcytes   (MGI Ref ID J:93896)
    • on a high fat diet oxygen consumption is increased 23% compared to wild-type mice   (MGI Ref ID J:93896)
  • muscle phenotype
  • dystonia
    • dystonic posturing is seen   (MGI Ref ID J:93896)
  • myoclonus
    • stimulus induced myoclonus is seen   (MGI Ref ID J:93896)
  • nervous system phenotype
  • abnormal neuron morphology
    • striatal neurons with reduced branches of neurites and occasional vacuoles within the neurons are seen   (MGI Ref ID J:93896)
  • gliosis
    • gliosis is seen associated with the spongiform lesions   (MGI Ref ID J:93896)
  • myoclonus
    • stimulus induced myoclonus is seen   (MGI Ref ID J:93896)
  • spongiform encephalopathy
    • spongiform lesions are seen predominantly in the striatum and to a lesser extent in the cortex   (MGI Ref ID J:93896)
    • the lesions are mostly associated with the white matter   (MGI Ref ID J:93896)
  • growth/size/body phenotype
  • decreased susceptibility to diet-induced obesity
    • on a high fat diet homozygous mutants are significantly leaner compared to wild-type mice   (MGI Ref ID J:93896)
  • liver/biliary system phenotype
  • decreased liver triglyceride level
    • triglyceride levels are lower in the livers of fasted mutant mice   (MGI Ref ID J:93896)

Ppargc1atm1Brsp/Ppargc1atm1Brsp

        Background Not Specified
  • cardiovascular system phenotype
  • congestive heart failure
    • TAC leads to accelerated cardiac dysfunction, accompanied by signs of heart failure   (MGI Ref ID J:111072)
  • increased heart weight
    • hearts from mutants subjected to transverse aortic constriction are more increased in weight and dilated than wild-type   (MGI Ref ID J:111072)
  • increased response of heart to induced stress
    • 2 months after transverse aortic constriction (TAC), develop profound cardiac dysfunction, with hearts becoming dilated and showing impaired ability to contract   (MGI Ref ID J:111072)
  • growth/size/body phenotype
  • cachexia
    • 2 months after TAC, mutants are visibly emaciated and show significant drops in weight, unlike wild-type   (MGI Ref ID J:111072)
  • respiratory system phenotype
  • increased lung weight
    • lungs are nearly quadrupled in weight 2 months after TAC as compared with only a moderate increase in wild-type TAC mice   (MGI Ref ID J:111072)
  • homeostasis/metabolism phenotype
  • increased response of heart to induced stress
    • 2 months after transverse aortic constriction (TAC), develop profound cardiac dysfunction, with hearts becoming dilated and showing impaired ability to contract   (MGI Ref ID J:111072)

Ppargc1atm1Brsp/Ppargc1atm1Brsp

        involves: 129
  • immune system phenotype
  • increased interleukin-6 secretion
View Research Applications

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

Cardiovascular Research
Heart Abnormalities
Metabolic Syndrome
Other

Developmental Biology Research
Embryonic Lethality (Homozygous)
      incomplete
Perinatal Lethality
      Homozygous

Diabetes and Obesity Research
Hyperglycemia
      diet-induced, moderate

Metabolism Research
Lipid Metabolism

Neurobiology Research
Behavioral and Learning Defects
Neurodegeneration

Research Tools
Diabetes and Obesity Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Ppargc1atm1Brsp
Allele Name targeted mutation 1, Bruce M Spiegelman
Allele Type Targeted (Null/Knockout)
Common Name(s) MKO; MKO-PGC-1alpha; PGC-1alpha-;
Mutation Made By Bruce Spiegelman,   Dana-Farber Cancer Institute
Strain of Origin129/Sv
ES Cell Line Strain129
Gene Symbol and Name Ppargc1a, peroxisome proliferative activated receptor, gamma, coactivator 1 alpha
Chromosome 5
Gene Common Name(s) A830037N07Rik; ENSMUSG00000079510; Gm11133; LEM6; PGC-1(alpha); PGC-1v; PGC1; PGC1A; PPAR Gamma Coactivator-1; PPAR gamma coactivator 1; PPARGC1; Pgc-1alpha; Pgc-1alphaa; Pgco1; RIKEN cDNA A830037N07 gene; predicted gene 11133; predicted gene, ENSMUSG00000079510;
Molecular Note This allele resulted from the crossing of Ppargc1atm2Brsp with mice expressing cre recombinase in the germline. Exons 3-5 were removed from the locus. Northern and Western blot analyses indicated a lack of transcript and protein in mutants. [MGI Ref ID J:93896]

Genotyping

Genotyping Information

Genotyping Protocols

Ppargc1asuptm1Brsp/sup, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Lin J; Wu PH; Tarr PT; Lindenberg KS; St-Pierre J; Zhang CY; Mootha VK; Jager S; Vianna CR; Reznick RM; Cui L; Manieri M; Donovan MX; Wu Z; Cooper MP; Fan MC; Rohas LM; Zavacki AM; Cinti S; Shulman GI; Lowell BB; Krainc D; Spiegelman BM. 2004. Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice. Cell 119(1):121-35. [PubMed: 15454086]  [MGI Ref ID J:93896]

Additional References

Ppargc1atm1Brsp related

Adhihetty PJ; Uguccioni G; Leick L; Hidalgo J; Pilegaard H; Hood DA. 2009. The role of PGC-1alpha on mitochondrial function and apoptotic susceptibility in muscle. Am J Physiol Cell Physiol 297(1):C217-25. [PubMed: 19439529]  [MGI Ref ID J:151162]

Agudelo LZ; Femenia T; Orhan F; Porsmyr-Palmertz M; Goiny M; Martinez-Redondo V; Correia JC; Izadi M; Bhat M; Schuppe-Koistinen I; Pettersson AT; Ferreira DM; Krook A; Barres R; Zierath JR; Erhardt S; Lindskog M; Ruas JL. 2014. Skeletal Muscle PGC-1alpha1 Modulates Kynurenine Metabolism and Mediates Resilience to Stress-Induced Depression. Cell 159(1):33-45. [PubMed: 25259918]  [MGI Ref ID J:213551]

Arany Z; Foo SY; Ma Y; Ruas JL; Bommi-Reddy A; Girnun G; Cooper M; Laznik D; Chinsomboon J; Rangwala SM; Baek KH; Rosenzweig A; Spiegelman BM. 2008. HIF-independent regulation of VEGF and angiogenesis by the transcriptional coactivator PGC-1alpha. Nature 451(7181):1008-12. [PubMed: 18288196]  [MGI Ref ID J:132657]

Arany Z; He H; Lin J; Hoyer K; Handschin C; Toka O; Ahmad F; Matsui T; Chin S; Wu PH; Rybkin II; Shelton JM; Manieri M; Cinti S; Schoen FJ; Bassel-Duby R; Rosenzweig A; Ingwall JS; Spiegelman BM. 2005. Transcriptional coactivator PGC-1 alpha controls the energy state and contractile function of cardiac muscle. Cell Metab 1(4):259-71. [PubMed: 16054070]  [MGI Ref ID J:129842]

Arany Z; Novikov M; Chin S; Ma Y; Rosenzweig A; Spiegelman BM. 2006. Transverse aortic constriction leads to accelerated heart failure in mice lacking PPAR-gamma coactivator 1alpha. Proc Natl Acad Sci U S A 103(26):10086-91. [PubMed: 16775082]  [MGI Ref ID J:111072]

Bhalla K; Hwang BJ; Dewi RE; Ou L; Twaddel W; Fang HB; Vafai SB; Vazquez F; Puigserver P; Boros L; Girnun GD. 2011. PGC1alpha Promotes Tumor Growth by Inducing Gene Expression Programs Supporting Lipogenesis. Cancer Res 71(21):6888-98. [PubMed: 21914785]  [MGI Ref ID J:177378]

Borniquel S; Garcia-Quintans N; Valle I; Olmos Y; Wild B; Martinez-Granero F; Soria E; Lamas S; Monsalve M. 2010. Inactivation of Foxo3a and subsequent downregulation of PGC-1 alpha mediate nitric oxide-induced endothelial cell migration. Mol Cell Biol 30(16):4035-44. [PubMed: 20547753]  [MGI Ref ID J:162779]

Bostrom P; Wu J; Jedrychowski MP; Korde A; Ye L; Lo JC; Rasbach KA; Bostrom EA; Choi JH; Long JZ; Kajimura S; Zingaretti MC; Vind BF; Tu H; Cinti S; Hojlund K; Gygi SP; Spiegelman BM. 2012. A PGC1-alpha-dependent myokine that drives brown-fat-like development of white fat and thermogenesis. Nature 481(7382):463-8. [PubMed: 22237023]  [MGI Ref ID J:181203]

Cherry AD; Suliman HB; Bartz RR; Piantadosi CA. 2014. Peroxisome proliferator-activated receptor gamma co-activator 1-alpha as a critical co-activator of the murine hepatic oxidative stress response and mitochondrial biogenesis in Staphylococcus aureus sepsis. J Biol Chem 289(1):41-52. [PubMed: 24253037]  [MGI Ref ID J:207184]

Chinsomboon J; Ruas J; Gupta RK; Thom R; Shoag J; Rowe GC; Sawada N; Raghuram S; Arany Z. 2009. The transcriptional coactivator PGC-1alpha mediates exercise-induced angiogenesis in skeletal muscle. Proc Natl Acad Sci U S A 106(50):21401-6. [PubMed: 19966219]  [MGI Ref ID J:155818]

Cooper MP; Uldry M; Kajimura S; Arany Z; Spiegelman BM. 2008. Modulation of PGC-1 coactivator pathways in brown fat differentiation through LRP130. J Biol Chem 283(46):31960-7. [PubMed: 18728005]  [MGI Ref ID J:143131]

Dougherty SE; Bartley AF; Lucas EK; Hablitz JJ; Dobrunz LE; Cowell RM. 2014. Mice lacking the transcriptional coactivator PGC-1alpha exhibit alterations in inhibitory synaptic transmission in the motor cortex. Neuroscience 271:137-48. [PubMed: 24769433]  [MGI Ref ID J:210606]

Egger A; Samardzija M; Sothilingam V; Tanimoto N; Lange C; Salatino S; Fang L; Garcia-Garrido M; Beck S; Okoniewski MJ; Neutzner A; Seeliger MW; Grimm C; Handschin C. 2012. PGC-1alpha determines light damage susceptibility of the murine retina. PLoS One 7(2):e31272. [PubMed: 22348062]  [MGI Ref ID J:185229]

Elias BC; Mathew S; Srichai MB; Palamuttam R; Bulus N; Mernaugh G; Singh AB; Sanders CR; Harris RC; Pozzi A; Zent R. 2014. The integrin beta1 subunit regulates paracellular permeability of kidney proximal tubule cells. J Biol Chem 289(12):8532-44. [PubMed: 24509849]  [MGI Ref ID J:212444]

Estall JL; Kahn M; Cooper MP; Fisher FM; Wu MK; Laznik D; Qu L; Cohen DE; Shulman GI; Spiegelman BM. 2009. Sensitivity of lipid metabolism and insulin signaling to genetic alterations in hepatic peroxisome proliferator-activated receptor-gamma coactivator-1alpha expression. Diabetes 58(7):1499-508. [PubMed: 19366863]  [MGI Ref ID J:154344]

Estall JL; Ruas JL; Choi CS; Laznik D; Badman M; Maratos-Flier E; Shulman GI; Spiegelman BM. 2009. PGC-1alpha negatively regulates hepatic FGF21 expression by modulating the heme/Rev-Erb(alpha) axis. Proc Natl Acad Sci U S A 106(52):22510-5. [PubMed: 20018698]  [MGI Ref ID J:156463]

Fabregat-Andres O; Tierrez A; Mata M; Estornell-Erill J; Ridocci-Soriano F; Monsalve M. 2011. Induction of PGC-1alpha expression can be detected in blood samples of patients with ST-segment elevation acute myocardial infarction. PLoS One 6(11):e26913. [PubMed: 22087236]  [MGI Ref ID J:180985]

Finley LW; Lee J; Souza A; Desquiret-Dumas V; Bullock K; Rowe GC; Procaccio V; Clish CB; Arany Z; Haigis MC. 2012. Skeletal muscle transcriptional coactivator PGC-1alpha mediates mitochondrial, but not metabolic, changes during calorie restriction. Proc Natl Acad Sci U S A 109(8):2931-6. [PubMed: 22308395]  [MGI Ref ID J:182618]

Guo X; Dason ES; Zanon-Moreno V; Jiang Q; Nahirnyj A; Chan D; Flanagan JG; Sivak JM. 2014. PGC-1alpha Signaling Coordinates Susceptibility to Metabolic and Oxidative Injury in the Inner Retina. Am J Pathol 184(4):1017-29. [PubMed: 24508229]  [MGI Ref ID J:208041]

Haase TN; Ringholm S; Leick L; Bienso RS; Kiilerich K; Johansen S; Nielsen MM; Wojtaszewski JF; Hidalgo J; Pedersen PA; Pilegaard H. 2011. Role of PGC-1alpha in exercise and fasting-induced adaptations in mouse liver. Am J Physiol Regul Integr Comp Physiol 301(5):R1501-9. [PubMed: 21832205]  [MGI Ref ID J:178809]

Handschin C; Chin S; Li P; Liu F; Maratos-Flier E; Lebrasseur NK; Yan Z; Spiegelman BM. 2007. Skeletal muscle fiber-type switching, exercise intolerance, and myopathy in PGC-1alpha muscle-specific knock-out animals. J Biol Chem 282(41):30014-21. [PubMed: 17702743]  [MGI Ref ID J:126742]

Handschin C; Choi CS; Chin S; Kim S; Kawamori D; Kurpad AJ; Neubauer N; Hu J; Mootha VK; Kim YB; Kulkarni RN; Shulman GI; Spiegelman BM. 2007. Abnormal glucose homeostasis in skeletal muscle-specific PGC-1alpha knockout mice reveals skeletal muscle-pancreatic beta cell crosstalk. J Clin Invest 117(11):3463-74. [PubMed: 17932564]  [MGI Ref ID J:127403]

Handschin C; Lin J; Rhee J; Peyer AK; Chin S; Wu PH; Meyer UA; Spiegelman BM. 2005. Nutritional regulation of hepatic heme biosynthesis and porphyria through PGC-1alpha. Cell 122(4):505-15. [PubMed: 16122419]  [MGI Ref ID J:115197]

Hasumi H; Baba M; Hasumi Y; Huang Y; Oh H; Hughes RM; Klein ME; Takikita S; Nagashima K; Schmidt LS; Linehan WM. 2012. Regulation of mitochondrial oxidative metabolism by tumor suppressor FLCN. J Natl Cancer Inst 104(22):1750-64. [PubMed: 23150719]  [MGI Ref ID J:192024]

Kiilerich K; Adser H; Jakobsen AH; Pedersen PA; Hardie DG; Wojtaszewski JF; Pilegaard H. 2010. PGC-1{alpha} increases PDH content but does not change acute PDH regulation in mouse skeletal muscle. Am J Physiol Regul Integr Comp Physiol 299(5):R1350-9. [PubMed: 20720174]  [MGI Ref ID J:165597]

Leick L; Hellsten Y; Fentz J; Lyngby SS; Wojtaszewski JF; Hidalgo J; Pilegaard H. 2009. PGC-1alpha mediates exercise-induced skeletal muscle VEGF expression in mice. Am J Physiol Endocrinol Metab 297(1):E92-103. [PubMed: 19401459]  [MGI Ref ID J:151197]

Leick L; Lyngby SS; Wojtasewski JF; Pilegaard H. 2010. PGC-1alpha is required for training-induced prevention of age-associated decline in mitochondrial enzymes in mouse skeletal muscle. Exp Gerontol 45(5):336-42. [PubMed: 20085804]  [MGI Ref ID J:164151]

Leick L; Wojtaszewski JF; Johansen ST; Kiilerich K; Comes G; Hellsten Y; Hidalgo J; Pilegaard H. 2008. PGC-1alpha is not mandatory for exercise- and training-induced adaptive gene responses in mouse skeletal muscle. Am J Physiol Endocrinol Metab 294(2):E463-74. [PubMed: 18073319]  [MGI Ref ID J:133426]

Li S; Arning E; Liu C; Vitvitsky V; Hernandez C; Banerjee R; Bottiglieri T; Lin JD. 2009. Regulation of homocysteine homeostasis through the transcriptional coactivator PGC-1alpha. Am J Physiol Endocrinol Metab 296(3):E543-8. [PubMed: 19158324]  [MGI Ref ID J:146959]

Liu C; Li S; Liu T; Borjigin J; Lin JD. 2007. Transcriptional coactivator PGC-1alpha integrates the mammalian clock and energy metabolism. Nature 447(7143):477-81. [PubMed: 17476214]  [MGI Ref ID J:122764]

Lucas EK; Dougherty SE; McMeekin LJ; Trinh AT; Reid CS; Cowell RM. 2012. Developmental alterations in motor coordination and medium spiny neuron markers in mice lacking pgc-1alpha. PLoS One 7(8):e42878. [PubMed: 22916173]  [MGI Ref ID J:190053]

Lucas EK; Markwardt SJ; Gupta S; Meador-Woodruff JH; Lin JD; Overstreet-Wadiche L; Cowell RM. 2010. Parvalbumin deficiency and GABAergic dysfunction in mice lacking PGC-1alpha. J Neurosci 30(21):7227-35. [PubMed: 20505089]  [MGI Ref ID J:160823]

Ma D; Li S; Lucas EK; Cowell RM; Lin JD. 2010. Neuronal inactivation of peroxisome proliferator-activated receptor gamma coactivator 1alpha (PGC-1alpha) protects mice from diet-induced obesity and leads to degenerative lesions. J Biol Chem 285(50):39087-95. [PubMed: 20947495]  [MGI Ref ID J:167589]

McDermott-Roe C; Ye J; Ahmed R; Sun XM; Serafin A; Ware J; Bottolo L; Muckett P; Canas X; Zhang J; Rowe GC; Buchan R; Lu H; Braithwaite A; Mancini M; Hauton D; Marti R; Garcia-Arumi E; Hubner N; Jacob H; Serikawa T; Zidek V; Papousek F; Kolar F; Cardona M; Ruiz-Meana M; Garcia-Dorado D; Comella JX; Felkin LE; Barton PJ; Arany Z; Pravenec M; Petretto E; Sanchis D; Cook SA. 2011. Endonuclease G is a novel determinant of cardiac hypertrophy and mitochondrial function. Nature 478(7367):114-8. [PubMed: 21979051]  [MGI Ref ID J:177433]

Morita M; Gravel SP; Chenard V; Sikstrom K; Zheng L; Alain T; Gandin V; Avizonis D; Arguello M; Zakaria C; McLaughlan S; Nouet Y; Pause A; Pollak M; Gottlieb E; Larsson O; St-Pierre J; Topisirovic I; Sonenberg N. 2013. mTORC1 controls mitochondrial activity and biogenesis through 4E-BP-dependent translational regulation. Cell Metab 18(5):698-711. [PubMed: 24206664]  [MGI Ref ID J:206172]

Olesen J; Ringholm S; Nielsen MM; Brandt CT; Pedersen JT; Halling JF; Goodyear LJ; Pilegaard H. 2013. Role of PGC-1alpha in exercise training- and resveratrol-induced prevention of age-associated inflammation. Exp Gerontol 48(11):1274-84. [PubMed: 23916840]  [MGI Ref ID J:210207]

Potthoff MJ; Boney-Montoya J; Choi M; He T; Sunny NE; Satapati S; Suino-Powell K; Xu HE; Gerard RD; Finck BN; Burgess SC; Mangelsdorf DJ; Kliewer SA. 2011. FGF15/19 regulates hepatic glucose metabolism by inhibiting the CREB-PGC-1alpha pathway. Cell Metab 13(6):729-38. [PubMed: 21641554]  [MGI Ref ID J:176084]

Ringholm S; Grunnet Knudsen J; Leick L; Lundgaard A; Munk Nielsen M; Pilegaard H. 2013. PGC-1alpha is required for exercise- and exercise training-induced UCP1 up-regulation in mouse white adipose tissue. PLoS One 8(5):e64123. [PubMed: 23717545]  [MGI Ref ID J:200834]

Ringholm S; Olesen J; Pedersen JT; Brandt CT; Halling JF; Hellsten Y; Prats C; Pilegaard H. 2013. Effect of lifelong resveratrol supplementation and exercise training on skeletal muscle oxidative capacity in aging mice; impact of PGC-1alpha. Exp Gerontol 48(11):1311-8. [PubMed: 23994519]  [MGI Ref ID J:210202]

Saint-Geniez M; Jiang A; Abend S; Liu L; Sweigard H; Connor KM; Arany Z. 2013. PGC-1alpha regulates normal and pathological angiogenesis in the retina. Am J Pathol 182(1):255-65. [PubMed: 23141926]  [MGI Ref ID J:192235]

Sawada N; Jiang A; Takizawa F; Safdar A; Manika A; Tesmenitsky Y; Kang KT; Bischoff J; Kalwa H; Sartoretto JL; Kamei Y; Benjamin LE; Watada H; Ogawa Y; Higashikuni Y; Kessinger CW; Jaffer FA; Michel T; Sata M; Croce K; Tanaka R; Arany Z. 2014. Endothelial PGC-1alpha mediates vascular dysfunction in diabetes. Cell Metab 19(2):246-58. [PubMed: 24506866]  [MGI Ref ID J:210653]

Selesniemi K; Lee HJ; Muhlhauser A; Tilly JL. 2011. From the Cover: Prevention of maternal aging-associated oocyte aneuploidy and meiotic spindle defects in mice by dietary and genetic strategies. Proc Natl Acad Sci U S A 108(30):12319-24. [PubMed: 21730149]  [MGI Ref ID J:174362]

St-Pierre J; Drori S; Uldry M; Silvaggi JM; Rhee J; Jager S; Handschin C; Zheng K; Lin J; Yang W; Simon DK; Bachoo R; Spiegelman BM. 2006. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell 127(2):397-408. [PubMed: 17055439]  [MGI Ref ID J:144665]

Stein S; Lohmann C; Handschin C; Stenfeldt E; Boren J; Luscher TF; Matter CM. 2010. ApoE-/- PGC-1alpha-/- mice display reduced IL-18 levels and do not develop enhanced atherosclerosis. PLoS One 5(10):e13539. [PubMed: 21042583]  [MGI Ref ID J:166708]

Summermatter S; Santos G; Perez-Schindler J; Handschin C. 2013. Skeletal muscle PGC-1alpha controls whole-body lactate homeostasis through estrogen-related receptor alpha-dependent activation of LDH B and repression of LDH A. Proc Natl Acad Sci U S A 110(21):8738-43. [PubMed: 23650363]  [MGI Ref ID J:197316]

Wrann CD; White JP; Salogiannnis J; Laznik-Bogoslavski D; Wu J; Ma D; Lin JD; Greenberg ME; Spiegelman BM. 2013. Exercise induces hippocampal BDNF through a PGC-1alpha/FNDC5 pathway. Cell Metab 18(5):649-59. [PubMed: 24120943]  [MGI Ref ID J:206072]

Wu J; Ruas JL; Estall JL; Rasbach KA; Choi JH; Ye L; Bostrom P; Tyra HM; Crawford RW; Campbell KP; Rutkowski DT; Kaufman RJ; Spiegelman BM. 2011. The unfolded protein response mediates adaptation to exercise in skeletal muscle through a PGC-1alpha/ATF6alpha complex. Cell Metab 13(2):160-9. [PubMed: 21284983]  [MGI Ref ID J:169564]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX10

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice can be bred as homozygotes, however approximately 50% of homozygotes exhibit postnatal lethality. The Donating Investigator reports maintaining homozygous pups at a higher temperature (77°F) increases their survival; heterozygote X heterozygote crosses yields less than the expected Mendelian ratio (the observed ratio is 1 homozygote in 8); and homozygous mice are more likely to have litters disappear or die after birth.
Mating System+/+ sibling x Heterozygote         (Female x Male)   08-AUG-09
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 $239.00Female or MaleHeterozygous for Ppargc1atm1Brsp  
Price per Pair (US dollars $)Pair Genotype
$311.00Heterozygous for Ppargc1atm1Brsp x Wild-type for Ppargc1atm1Brsp  
$311.00Wild-type for Ppargc1atm1Brsp x Heterozygous for Ppargc1atm1Brsp  

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 $310.70Female or MaleHeterozygous for Ppargc1atm1Brsp  
Price per Pair (US dollars $)Pair Genotype
$404.30Heterozygous for Ppargc1atm1Brsp x Wild-type for Ppargc1atm1Brsp  
$404.30Wild-type for Ppargc1atm1Brsp x Heterozygous for Ppargc1atm1Brsp  

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
   101043 B6129SF1/J (approximate)
   000664 C57BL/6J
 
  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.


(6.8)