Strain Name:

B6.129S1-Thrbtm1Df/J

Stock Number:

003462

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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

Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6
Donor Strain 129S1 via W9.5 ES (+Kitl-SlJ) ES cell line
 
Donating InvestigatorDr. Douglas Forrest,   National Institutes of Health

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Thrbtm1Df targeted mutation are viable and fertile displaying normal growth rates. Homozygous mutant mice exhibit goiter and elevated levels of both thyroid hormone and thyroid stimulating hormone. Defects in liver responses to thyroid hormone and subtle behavioral abnormalities are observed. The mice fail to develop normal hearing, as assessed by impaired auditory-evoked brainstem responses, and are susceptible to audiogenic seizures. This strain provides a recessive model for the human syndrome of generalized thyroid hormone resistance (GTHR).

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Thyroid Hormone Resistance, Generalized, Autosomal Recessive; GRTH
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Thyroid Hormone Resistance, Generalized, Autosomal Dominant; GRTH   (THRB)
Thyroid Hormone Resistance, Selective Pituitary; PRTH   (THRB)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Thrbtm1Df/Thrbtm1Df

        B6.129S1-Thrbtm1Df
  • hearing/vestibular/ear phenotype
  • abnormal cochlear inner hair cell physiology
    • at P19, homozygotes display absence of the fast-activating potassium current IK,f which is associated with IHC maturation and is normally detected by P14 and rises to plateau after P20   (MGI Ref ID J:118402)
  • increased or absent threshold for auditory brainstem response
    • adult homozygotes exhibit significantly increased ABR thresholds for all test stimuli (click, 8-, 16-, and 32-kHz), with waveforms detected only in response to 85 dB SPL vs 40-45 dB SPL in wild-type mice   (MGI Ref ID J:118402)
  • nervous system phenotype
  • abnormal cochlear inner hair cell physiology
    • at P19, homozygotes display absence of the fast-activating potassium current IK,f which is associated with IHC maturation and is normally detected by P14 and rises to plateau after P20   (MGI Ref ID J:118402)

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

Thrbtm1Df/Thrb+

        either: B6.129S1-Thrbtm1Df or (involves: 129S1/Sv * C57BL/6J)
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • unlike homozygotes, heterozygotes of either genetic background are resistant to audiogenic seizures   (MGI Ref ID J:103702)

Thrbtm1Df/Thrbtm1Df

        involves: 129S1/Sv * C57BL/6J
  • endocrine/exocrine gland phenotype
  • abnormal thyroid follicle morphology
    • number and size of follicles both increased   (MGI Ref ID J:34421)
  • enlarged thyroid gland
    • 1.5-2 fold increase in thyroid size   (MGI Ref ID J:34421)
    • thyroid gland hyperplasia   (MGI Ref ID J:34421)
  • increased activity of thyroid
    • hyperactive state with increased epithelial cell turnover   (MGI Ref ID J:34421)
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • homozygotes behave normally in learning tests such as the Morris water task and in contextual fear conditioning as well as in open field and Y maze tests mice behave normally in learning tests such as the Morris water task and in contextual fear conditioning as well as in open field and Y maze tests   (MGI Ref ID J:34421)
    • adult homozygotes do not display circling or any other behavioral or neuroanatomical defects   (MGI Ref ID J:63101)
  • nervous system phenotype
  • abnormal cochlear inner hair cell physiology
    • homozygotes exhibit a delay in the induction of the fast-activating potassium current IK,f which is associated with IHC maturation and is normally induced by P13 and plateaus after P20   (MGI Ref ID J:73382)
    • IK,f is largely absent at P15-P18, when hearing impairment is first evident in young homozygotes   (MGI Ref ID J:118178)
    • IK,f eventually appears with a significant delay and reaches half-maximal expression at ~P28   (MGI Ref ID J:118178)
    • at P50, IK,f approaches magnitudes detected in wild-type IHCs   (MGI Ref ID J:118178)
    • despite retarded expression of IK,f, development of the endocochlear potential, other hair cell transducer conductances, and OHC electromotility, appear normal   (MGI Ref ID J:118178)
    • in neonatal mutant IHCs, expression of the fast voltage-activated conductance starts ~7 days later and requires >1 month to reach adult wild-type levels   (MGI Ref ID J:48666)
  • abnormal cochlear outer hair cell physiology   (MGI Ref ID J:73382)
    • decreased cochlear outer hair cell electromotility
      • at P8, homozygotes exhibit a slight impairment of electromechanical transduction in OHCs, as shown by slightly reduced nonlinear capacitance (150 46 fF/pF) relative to wild-type mice (290 47 fF/pF) or mice that are double homozygous for for Thratm1Ven and Thrbtm1Df (87 32 fF/pF)   (MGI Ref ID J:73382)
      • at P9, homozygotes exhibit a nonlinear capacitance of 271 27 fF/pF, suggesting that differences in voltage-dependent capacitance of mutant OHCs at P8 are not functionally significant   (MGI Ref ID J:118178)
  • short cochlear outer hair cells
    • at P8, mutant OHCs are smaller than wild-type OHCs, based on linear, voltage-independent capacitances   (MGI Ref ID J:118178)
  • hearing/vestibular/ear phenotype
  • abnormal cochlear inner hair cell physiology
    • homozygotes exhibit a delay in the induction of the fast-activating potassium current IK,f which is associated with IHC maturation and is normally induced by P13 and plateaus after P20   (MGI Ref ID J:73382)
    • IK,f is largely absent at P15-P18, when hearing impairment is first evident in young homozygotes   (MGI Ref ID J:118178)
    • IK,f eventually appears with a significant delay and reaches half-maximal expression at ~P28   (MGI Ref ID J:118178)
    • at P50, IK,f approaches magnitudes detected in wild-type IHCs   (MGI Ref ID J:118178)
    • despite retarded expression of IK,f, development of the endocochlear potential, other hair cell transducer conductances, and OHC electromotility, appear normal   (MGI Ref ID J:118178)
    • in neonatal mutant IHCs, expression of the fast voltage-activated conductance starts ~7 days later and requires >1 month to reach adult wild-type levels   (MGI Ref ID J:48666)
  • abnormal cochlear outer hair cell physiology   (MGI Ref ID J:73382)
    • decreased cochlear outer hair cell electromotility
      • at P8, homozygotes exhibit a slight impairment of electromechanical transduction in OHCs, as shown by slightly reduced nonlinear capacitance (150 46 fF/pF) relative to wild-type mice (290 47 fF/pF) or mice that are double homozygous for for Thratm1Ven and Thrbtm1Df (87 32 fF/pF)   (MGI Ref ID J:73382)
      • at P9, homozygotes exhibit a nonlinear capacitance of 271 27 fF/pF, suggesting that differences in voltage-dependent capacitance of mutant OHCs at P8 are not functionally significant   (MGI Ref ID J:118178)
  • abnormal organ of Corti morphology
    • at P9, inner sulcus differentiation is delayed, and the tunnel of Corti remains unopened   (MGI Ref ID J:73382)
    • at P20, the inner sulcus has opened, but the undelying epithelium is slightly thicker than normal   (MGI Ref ID J:73382)
    • short cochlear outer hair cells
      • at P8, mutant OHCs are smaller than wild-type OHCs, based on linear, voltage-independent capacitances   (MGI Ref ID J:118178)
  • abnormal tectorial membrane morphology
    • ultrastructurally, adult homozygotes display a mild disorganization of the striated sheet matrix only in the upper regions of the tectorial membrane   (MGI Ref ID J:73382)
    • enlarged tectorial membrane
      • at P9, the tectorial membrane is slightly enlarged although not to the extent observed in mice that are double homozygous for Thratm1Ven and Thrbtm1Df   (MGI Ref ID J:73382)
      • at P20, the tectorial membrane remains slightly enlarged, although it extends to the hair cells and is not grossly misshapen as in mice that are double homozygous for Thratm1Ven and Thrbtm1Df   (MGI Ref ID J:73382)
  • deafness
    • homozygotes exhibit a profound hearing loss across a wide range of frequencies   (MGI Ref ID J:63101)
  • delayed inner ear development
    • at 15 weeks, adult homozygotes display normal inner ear innervation with no major hypothyroid-like defects in the sensorineural epithelium, tectorial membrane, stria vascularis, or spiral ganglion   (MGI Ref ID J:63101)
    • despite an absence of gross cochlear malformations, adult homozygotes exhibit a delay in early postnatal development of the organ of Corti, that is milder than that observed in mice that are double homozygous for Thratm1Ven and Thrbtm1Df   (MGI Ref ID J:73382)
  • increased or absent threshold for auditory brainstem response
    • at 9-15 weeks, adult homozygotes show significantly elevated ABR thresholds for a click stimulus (1-16 kHz) and for all test pure tones (8, 16, 32 kHz)   (MGI Ref ID J:63101)
    • ~95% of adult homozygotes exhibit ABR thresholds in the 70-100 dB SPL range, while 5% display thresholds in the upper limit of the normal range   (MGI Ref ID J:63101)
    • ~10% of adult homozygotes are unresponsive to any stimulus frequency tested at 100 SPL   (MGI Ref ID J:63101)
    • young homozygous mutant progeny (P18-P28) display significantly elevated ABR thresholds, independent of the maternal genotype (i.e. homozygous (hyperthyroid) vs heterozygous (euthyroid) mutant mothers)   (MGI Ref ID J:63101)
    • although ABR responses are diminished in amplitude, ABR waveforms display a normal pattern of peaks, suggesting that the defect resides in the primary response of the cochlea   (MGI Ref ID J:63101)
    • at 2-3 months, homozygotes exhibit significantly elevated ABR thresholds for click, 8-, 16-, and 32-kHz frequency stimuli relative to wild-type or heterozygous littermates   (MGI Ref ID J:118178)
  • homeostasis/metabolism phenotype
  • decreased circulating thyroxine level
    • T4 levels are reduced at 1.5 years of age   (MGI Ref ID J:34421)
  • increased circulating thyroxine level
    • elevated serum levels of T4 between 5 and 40 weeks of age   (MGI Ref ID J:34421)
  • increased circulating triiodothyronine level
    • serum T3 levels elevated   (MGI Ref ID J:34421)
  • increased thyroid-stimulating hormone level
    • elevated TSH levels are present although no morphological abnormalities are seen in the pituitary   (MGI Ref ID J:34421)

Thrbtm1Df/Thrbtm1Df

        either: B6.129S1-Thrbtm1Df or (involves: 129S1/Sv * C57BL/6J)
  • behavior/neurological phenotype
  • audiogenic seizures
    • on a mixed genetic background, ~100% of homozygotes display early-onset and persistent auditory seizure susceptibility (AGS) after P16 (but not prior to P11) up to >6 months of age, whereas ~27% of background-matched wild-type show AGS up to 4 weeks but not at 10 months of age   (MGI Ref ID J:103702)
    • on a congenic C57BL/6J background, 100% of homozygotes exhibit AGS relative to 0% in congenic wild-type mice   (MGI Ref ID J:103702)
  • hearing/vestibular/ear phenotype
  • deafness
    • at 2 months, homozygotes exhibit residual ABR responses with slightly diminished waveforms, indicating that deafness is severe but not complete   (MGI Ref ID J:103702)
  • increased or absent threshold for auditory brainstem response
    • at 2 months, homozygotes exhibit significantly increased ABR thresholds for all test stimuli (click, 8-, 16-, and 32-kHz) relative to wild-type littermates   (MGI Ref ID J:103702)
  • homeostasis/metabolism phenotype
  • increased circulating thyroxine level
    • homozygotes exhibit a ~2-fold increase in total serum T4 levels relative to wild-type mice   (MGI Ref ID J:103702)
  • increased circulating triiodothyronine level
    • homozygotes exhibit a ~2-fold increase in total serum T3 levels relative to wild-type mice   (MGI Ref ID J:103702)
  • increased thyroid-stimulating hormone level
    • homozygotes exhibit a ~2-fold increase in total serum TSH levels relative to wild-type mice   (MGI Ref ID J:103702)
  • nervous system phenotype
  • audiogenic seizures
    • on a mixed genetic background, ~100% of homozygotes display early-onset and persistent auditory seizure susceptibility (AGS) after P16 (but not prior to P11) up to >6 months of age, whereas ~27% of background-matched wild-type show AGS up to 4 weeks but not at 10 months of age   (MGI Ref ID J:103702)
    • on a congenic C57BL/6J background, 100% of homozygotes exhibit AGS relative to 0% in congenic wild-type mice   (MGI Ref ID J:103702)

Thrbtm1Df/Thrbtm1Df

        involves: 129S1/Sv * C57BL/6J * FVB/NJ
  • hearing/vestibular/ear phenotype
  • abnormal cochlear nerve compound action potential
    • elevated compound action potential thresholds at all frequencies tested in mice at 3 - 4 months of age   (MGI Ref ID J:145941)
    • however, the shape of the compound action potential waveform is similar to controls   (MGI Ref ID J:145941)
  • abnormal distortion product otoacoustic emission
    • DPOAE thresholds are altered with no 2f1-f2 amplitude baseline at an f2 frequency of 16 kHz and significantly elevated thresholds at frequencies greater than 8 kHz   (MGI Ref ID J:145941)
  • decreased cochlear microphonics
    • severely reduced   (MGI Ref ID J:145941)
  • enlarged tectorial membrane
    • thickened and enlarged, most prominently in the apical turns   (MGI Ref ID J:145941)
  • increased or absent threshold for auditory brainstem response
    • ABR thresholds for click auditory stimuli are increased in mice at 3 - 4 months of age   (MGI Ref ID J:145941)
  • nervous system phenotype
  • abnormal cochlear nerve compound action potential
    • elevated compound action potential thresholds at all frequencies tested in mice at 3 - 4 months of age   (MGI Ref ID J:145941)
    • however, the shape of the compound action potential waveform is similar to controls   (MGI Ref ID J:145941)
  • decreased cochlear microphonics
    • severely reduced   (MGI Ref ID J:145941)
View Research Applications

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

Thrbtm1Df related

Endocrine Deficiency Research
Hypothalamus/Pituitary Defects
Thyroid Defects
      goiter
      hyperthyroidism

Internal/Organ Research
Liver Defects
Thyroid Defects
      hyperthyroidism

Neurobiology Research
Epilepsy
      audiogenic seizures
Hearing Defects

Research Tools
Endocrine Deficiency Research
Genetics Research
      Mutagenesis and Transgenesis
Sensorineural Research

Sensorineural Research
Eye Defects
Hearing Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Thrbtm1Df
Allele Name targeted mutation 1, Douglas Forrest
Allele Type Targeted (Null/Knockout)
Common Name(s) TRbeta-; Thrb-;
Mutation Made ByDr. Douglas Forrest,   National Institutes of Health
Strain of Origin129S1/Sv-Oca2<+> Tyr<+> Kitl<+>
ES Cell Line NameW9.5/W95
ES Cell Line Strain129S1/Sv-Oca2<+> Tyr<+> Kitl<+>
Gene Symbol and Name Thrb, thyroid hormone receptor beta
Chromosome 14
Gene Common Name(s) C-ERBA-2; C-ERBA-BETA; ERBA2; GRTH; NR1A2; PRTH; RATT3REC; T3R[b]; T3Rbeta; T3rec; THR1; THRB1; THRB2; TR beta; TRbeta; Thrb1; Thrb2; c-erbAbeta; thyroid hormone receptor beta 1; thyroid hormone receptor beta 2;
Molecular Note Insertion of a neomycin cassette into exon 3, disrupts both the beta1 and beta2 isoforms of this gene. RT-PCR analysis identified a short transcript in homozygous mutant mice. Upon sequencing, this transcript revealed a deletion of exon 3 sequences, and fused beta1 exon 2 to exon 4. This results in an aberrant open reading frame, which terminates early into exon 4. No functional protein is predicted from this transcript, as the essential DNA binding and T3 binding domains not present. [MGI Ref ID J:34421]

Genotyping

Genotyping Information

Genotyping Protocols

Thrbtm1Df, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Forrest D; Hanebuth E; Smeyne RJ; Everds N; Stewart CL; Wehner JM; Curran T. 1996. Recessive resistance to thyroid hormone in mice lacking thyroid hormone receptor beta: evidence for tissue-specific modulation of receptor function. EMBO J 15(12):3006-15. [PubMed: 8670802]  [MGI Ref ID J:34421]

Additional References

Amma LL; Campos-Barros A; Wang Z; Vennstrom B; Forrest D. 2001. Distinct Tissue-Specific Roles for Thyroid Hormone Receptors beta and alpha1 in Regulation of Type 1 Deiodinase Expression. Mol Endocrinol 15(3):467-75. [PubMed: 11222747]  [MGI Ref ID J:67946]

Forrest D; Erway LC; Ng L; Altschuler R; Curran T. 1996. Thyroid hormone receptor beta is essential for development of auditory function. Nat Genet 13(3):354-7. [PubMed: 8673137]  [MGI Ref ID J:63101]

Forrest D; Vennstrom B. 2000. Functions of thyroid hormone receptors in mice Thyroid 10(1):41-52. [PubMed: 10691312]  [MGI Ref ID J:60449]

Gullberg H; Rudling M; Salto C; Forrest D; Angelin B; Vennstrom B. 2002. Requirement for thyroid hormone receptor beta in T3 regulation of cholesterol metabolism in mice. Mol Endocrinol 16(8):1767-77. [PubMed: 12145333]  [MGI Ref ID J:89791]

Johansson C; Gothe S; Forrest D; Vennstrom B; Thoren P. 1999. Cardiovascular phenotype and temperature control in mice lacking thyroid hormone receptor-beta or both alpha1 and beta. Am J Physiol 276(6 Pt 2):H2006-12. [PubMed: 10362681]  [MGI Ref ID J:55864]

Mansen A; Yu F; Forrest D; Larsson L; Vennstrom B. 2001. TRs have common and isoform-specific functions in regulation of the cardiac myosin heavy chain genes. Mol Endocrinol 15(12):2106-14. [PubMed: 11731612]  [MGI Ref ID J:72960]

Salto C; Kindblom JM; Johansson C; Wang Z; Gullberg H; Nordstrom K; Mansen A; Ohlsson C; Thoren P; Forrest D; Vennstrom B. 2001. Ablation of TRalpha2 and a Concomitant Overexpression of alpha1 Yields a Mixed Hypo- and Hyperthyroid Phenotype in Mice. Mol Endocrinol 15(12):2115-28. [PubMed: 11731613]  [MGI Ref ID J:72959]

Thrbtm1Df related

Abel ED; Ahima RS; Boers ME; Elmquist JK; Wondisford FE. 2001. Critical role for thyroid hormone receptor beta2 in the regulation of paraventricular thyrotropin-releasing hormone neurons. J Clin Invest 107(8):1017-23. [PubMed: 11306605]  [MGI Ref ID J:68885]

Amma LL; Campos-Barros A; Wang Z; Vennstrom B; Forrest D. 2001. Distinct Tissue-Specific Roles for Thyroid Hormone Receptors beta and alpha1 in Regulation of Type 1 Deiodinase Expression. Mol Endocrinol 15(3):467-75. [PubMed: 11222747]  [MGI Ref ID J:67946]

Amma LL; Goodyear R; Faris JS; Jones I; Ng L; Richardson G; Forrest D. 2003. An emilin family extracellular matrix protein identified in the cochlear basilar membrane. Mol Cell Neurosci 23(3):460-72. [PubMed: 12837629]  [MGI Ref ID J:126300]

Barros AC; Erway LC; Krezel W; Curran T; Kastner P; Chambon P; Forrest D. 1998. Absence of thyroid hormone receptor beta-retinoid X receptor interactions in auditory function and in the pituitary-thyroid axis. Neuroreport 9(13):2933-7. [PubMed: 9804293]  [MGI Ref ID J:103717]

Bassett JH; Nordstrom K; Boyde A; Howell PG; Kelly S; Vennstrom B; Williams GR. 2007. Thyroid Status during Skeletal Development Determines Adult Bone Structure and Mineralization. Mol Endocrinol 21(8):1893-1904. [PubMed: 17488972]  [MGI Ref ID J:123118]

Calza L; Forrest D; Vennstrom B; Hokfelt T. 2000. Expression of peptides and other neurochemical markers in hypothalamus and olfactory bulb of mice devoid of all known thyroid hormone receptors. Neuroscience 101(4):1001-12. [PubMed: 11113349]  [MGI Ref ID J:118024]

Contreras-Jurado C; Garcia-Serrano L; Gomez-Ferreria M; Costa C; Paramio JM; Aranda A. 2011. The thyroid hormone receptors as modulators of skin proliferation and inflammation. J Biol Chem 286(27):24079-88. [PubMed: 21566120]  [MGI Ref ID J:175277]

Cordas EA; Ng L; Hernandez A; Kaneshige M; Cheng SY; Forrest D. 2012. Thyroid hormone receptors control developmental maturation of the middle ear and the size of the ossicular bones. Endocrinology 153(3):1548-60. [PubMed: 22253431]  [MGI Ref ID J:182543]

Dellovade TL; Chan J; Vennstrom B; Forrest D; Pfaff DW. 2000. The two thyroid hormone receptor genes have opposite effects on estrogen-stimulated sex behaviors. Nat Neurosci 3(5):472-5. [PubMed: 10769387]  [MGI Ref ID J:61839]

Dettling J; Franz C; Zimmermann U; Lee SC; Bress A; Brandt N; Feil R; Pfister M; Engel J; Flamant F; Ruttiger L; Knipper M. 2014. Autonomous functions of murine thyroid hormone receptor TRalpha and TRbeta in cochlear hair cells. Mol Cell Endocrinol 382(1):26-37. [PubMed: 24012852]  [MGI Ref ID J:208736]

Dkhissi-Benyahya O; Gronfier C; De Vanssay W; Flamant F; Cooper HM. 2007. Modeling the role of mid-wavelength cones in circadian responses to light. Neuron 53(5):677-87. [PubMed: 17329208]  [MGI Ref ID J:128662]

Forrest D; Erway LC; Ng L; Altschuler R; Curran T. 1996. Thyroid hormone receptor beta is essential for development of auditory function. Nat Genet 13(3):354-7. [PubMed: 8673137]  [MGI Ref ID J:63101]

Furumoto H; Ying H; Chandramouli GV; Zhao L; Walker RL; Meltzer PS; Willingham MC; Cheng SY. 2005. An unliganded thyroid hormone beta receptor activates the cyclin D1/cyclin-dependent kinase/retinoblastoma/E2F pathway and induces pituitary tumorigenesis. Mol Cell Biol 25(1):124-35. [PubMed: 15601836]  [MGI Ref ID J:95391]

Garcia-Serrano L; Gomez-Ferreria MA; Contreras-Jurado C; Segrelles C; Paramio JM; Aranda A. 2011. The thyroid hormone receptors modulate the skin response to retinoids. PLoS One 6(8):e23825. [PubMed: 21858227]  [MGI Ref ID J:176352]

Golozoubova V; Gullberg H; Matthias A; Cannon B; Vennstrom B; Nedergaard J. 2004. Depressed thermogenesis but competent brown adipose tissue recruitment in mice devoid of all hormone-binding thyroid hormone receptors. Mol Endocrinol 18(2):384-401. [PubMed: 14630998]  [MGI Ref ID J:87747]

Gothe S; Wang Z; Ng L; Kindblom JM; Barros AC; Ohlsson C; Vennstrom B; Forrest D. 1999. Mice devoid of all known thyroid hormone receptors are viable but exhibit disorders of the pituitary-thyroid axis, growth, and bone maturation. Genes Dev 13(10):1329-41. [PubMed: 10346821]  [MGI Ref ID J:55414]

Gullberg H; Rudling M; Forrest D; Angelin B; Vennstrom B. 2000. Thyroid hormone receptor beta-deficient mice show complete loss of the normal cholesterol 7alpha-hydroxylase (CYP7A) response to thyroid hormone but display enhanced resistance to dietary cholesterol. Mol Endocrinol 14(11):1739-49. [PubMed: 11075809]  [MGI Ref ID J:125475]

Gullberg H; Rudling M; Salto C; Forrest D; Angelin B; Vennstrom B. 2002. Requirement for thyroid hormone receptor beta in T3 regulation of cholesterol metabolism in mice. Mol Endocrinol 16(8):1767-77. [PubMed: 12145333]  [MGI Ref ID J:89791]

Hashimoto K; Cohen RN; Yamada M; Markan KR; Monden T; Satoh T; Mori M; Wondisford FE. 2006. Cross-talk between thyroid hormone receptor and liver X receptor regulatory pathways is revealed in a thyroid hormone resistance mouse model. J Biol Chem 281(1):295-302. [PubMed: 16260782]  [MGI Ref ID J:105670]

Johansson C; Gothe S; Forrest D; Vennstrom B; Thoren P. 1999. Cardiovascular phenotype and temperature control in mice lacking thyroid hormone receptor-beta or both alpha1 and beta. Am J Physiol 276(6 Pt 2):H2006-12. [PubMed: 10362681]  [MGI Ref ID J:55864]

Johansson C; Lannergren J; Lunde PK; Vennstrom B; Thoren P; Westerblad H. 2000. Isometric force and endurance in soleus muscle of thyroid hormone receptor-alpha(1)- or -beta-deficient mice. Am J Physiol Regul Integr Comp Physiol 278(3):R598-603. [PubMed: 10712278]  [MGI Ref ID J:61289]

Johansson C; Lunde PK; Gothe S; Lannergren J; Westerblad H. 2003. Isometric force and endurance in skeletal muscle of mice devoid of all known thyroid hormone receptors. J Physiol 547(Pt 3):789-96. [PubMed: 12562961]  [MGI Ref ID J:105508]

Johnson KR; Zheng QY; Noben-Trauth K. 2006. Strain background effects and genetic modifiers of hearing in mice. Brain Res 1091(1):79-88. [PubMed: 16579977]  [MGI Ref ID J:110459]

Kapoor R; Ghosh H; Nordstrom K; Vennstrom B; Vaidya VA. 2011. Loss of thyroid hormone receptor beta is associated with increased progenitor proliferation and NeuroD positive cell number in the adult hippocampus. Neurosci Lett 487(2):199-203. [PubMed: 20959135]  [MGI Ref ID J:168644]

Kato Y; Ying H; Willingham MC; Cheng SY. 2004. A tumor suppressor role for thyroid hormone beta receptor in a mouse model of thyroid carcinogenesis. Endocrinology 145(10):4430-8. [PubMed: 15231697]  [MGI Ref ID J:92629]

Kindblom JM; Gevers EF; Skrtic SM; Lindberg MK; Gothe S; Tornell J; Vennstrom B; Ohlsson C. 2005. Increased adipogenesis in bone marrow but decreased bone mineral density in mice devoid of thyroid hormone receptors. Bone 36(4):607-16. [PubMed: 15780976]  [MGI Ref ID J:98068]

Kindblom JM; Kindblom JM; Gothe S; Forrest D; Tornell J; Tornell J; Vennstrom B; Ohlsson C. 2001. GH substitution reverses the growth phenotype but not the defective ossification in thyroid hormone receptor alpha1-/-beta-/- mice. J Endocrinol 171(1):15-22. [PubMed: 11572786]  [MGI Ref ID J:72045]

Knostman KA; Jhiang SM; Capen CC. 2007. Genetic alterations in thyroid cancer: the role of mouse models. Vet Pathol 44(1):1-14. [PubMed: 17197619]  [MGI Ref ID J:129329]

Lopez-Fontal R; Zeini M; Traves PG; Gomez-Ferreria M; Aranda A; Saez GT; Cerda C; Martin-Sanz P; Hortelano S; Bosca L. 2010. Mice lacking thyroid hormone receptor Beta show enhanced apoptosis and delayed liver commitment for proliferation after partial hepatectomy. PLoS One 5(1):e8710. [PubMed: 20090848]  [MGI Ref ID J:157232]

Mansen A; Yu F; Forrest D; Larsson L; Vennstrom B. 2001. TRs have common and isoform-specific functions in regulation of the cardiac myosin heavy chain genes. Mol Endocrinol 15(12):2106-14. [PubMed: 11731612]  [MGI Ref ID J:72960]

Ng L; Hernandez A; He W; Ren T; Srinivas M; Ma M; Galton VA; St Germain DL; Forrest D. 2009. A protective role for type 3 deiodinase, a thyroid hormone-inactivating enzyme, in cochlear development and auditory function. Endocrinology 150(4):1952-60. [PubMed: 19095741]  [MGI Ref ID J:151815]

Ng L; Pedraza PE; Faris JS; Vennstrom B; Curran T; Morreale de Escobar G; Forrest D. 2001. Audiogenic seizure susceptibility in thyroid hormone receptor beta-deficient mice. Neuroreport 12(11):2359-62. [PubMed: 11496110]  [MGI Ref ID J:103702]

Ng L; Rusch A; Amma LL; Nordstrom K; Erway LC; Vennstrom B; Forrest D. 2001. Suppression of the deafness and thyroid dysfunction in Thrb-null mice by an independent mutation in the Thra thyroid hormone receptor alpha gene. Hum Mol Genet 10(23):2701-8. [PubMed: 11726557]  [MGI Ref ID J:118402]

Onishi A; Peng GH; Chen S; Blackshaw S. 2010. Pias3-dependent SUMOylation controls mammalian cone photoreceptor differentiation. Nat Neurosci 13(9):1059-65. [PubMed: 20729845]  [MGI Ref ID J:165275]

Rusch A; Erway LC; Oliver D; Vennstrom B; Forrest D. 1998. Thyroid hormone receptor beta-dependent expression of a potassium conductance in inner hair cells at the onset of hearing. Proc Natl Acad Sci U S A 95(26):15758-62. [PubMed: 9861043]  [MGI Ref ID J:118178]

Rusch A; Erway LC; Vennstrom B; Forrest D. 1998. Thyroid hormone receptor Beta is involved in developmental maturation of inner hair cell ionic conductances Hered Deaf News 15:51.  [MGI Ref ID J:48666]

Rusch A; Ng L; Goodyear R; Oliver D; Lisoukov I; Vennstrom B; Richardson G; Kelley MW; Forrest D. 2001. Retardation of cochlear maturation and impaired hair cell function caused by deletion of all known thyroid hormone receptors. J Neurosci 21(24):9792-800. [PubMed: 11739587]  [MGI Ref ID J:73382]

Sandhofer C; Schwartz HL; Mariash CN; Forrest D; Oppenheimer JH. 1998. Beta receptor isoforms are not essential for thyroid hormone-dependent acceleration of PCP-2 and myelin basic protein gene expression in the developing brains of neonatal mice. Mol Cell Endocrinol 137(2):109-15. [PubMed: 9605512]  [MGI Ref ID J:47019]

Sjogren M; Alkemade A; Mittag J; Nordstrom K; Katz A; Rozell B; Westerblad H; Arner A; Vennstrom B. 2007. Hypermetabolism in mice caused by the central action of an unliganded thyroid hormone receptor alpha1. EMBO J 26(21):4535-45. [PubMed: 17932484]  [MGI Ref ID J:141474]

Suzuki H; Zhang XY; Forrest D; Willingham MC; Cheng SY. 2003. Marked potentiation of the dominant negative action of a mutant thyroid hormone receptor beta in mice by the ablation of one wild-type beta allele. Mol Endocrinol 17(5):895-907. [PubMed: 12576488]  [MGI Ref ID J:83199]

Tinnikov A; Nordstrom K; Thoren P; Kindblom JM; Malin S; Rozell B; Adams M; Rajanayagam O; Pettersson S; Ohlsson C; Chatterjee K; Vennstrom B. 2002. Retardation of post-natal development caused by a negatively acting thyroid hormone receptor alpha1. EMBO J 21(19):5079-87. [PubMed: 12356724]  [MGI Ref ID J:100290]

Vujovic M; Nordstrom K; Gauthier K; Flamant F; Visser TJ; Vennstrom B; Mittag J. 2009. Interference of a mutant thyroid hormone receptor alpha1 with hepatic glucose metabolism. Endocrinology 150(6):2940-7. [PubMed: 19282388]  [MGI Ref ID J:158178]

Wallis K; Sjogren M; van Hogerlinden M; Silberberg G; Fisahn A; Nordstrom K; Larsson L; Westerblad H; Morreale de Escobar G; Shupliakov O; Vennstrom B. 2008. Locomotor deficiencies and aberrant development of subtype-specific GABAergic interneurons caused by an unliganded thyroid hormone receptor alpha1. J Neurosci 28(8):1904-15. [PubMed: 18287507]  [MGI Ref ID J:131713]

Weiss RE; Forrest D; Pohlenz J; Cua K; Curran T; Refetoff S. 1997. Thyrotropin regulation by thyroid hormone in thyroid hormone receptor beta-deficient mice. Endocrinology 138(9):3624-9. [PubMed: 9275045]  [MGI Ref ID J:42911]

Winter H; Braig C; Zimmermann U; Geisler HS; Franzer JT; Weber T; Ley M; Engel J; Knirsch M; Bauer K; Christ S; Walsh EJ; McGee J; Kopschall I; Rohbock K; Knipper M. 2006. Thyroid hormone receptors TRalpha1 and TRbeta differentially regulate gene expression of Kcnq4 and prestin during final differentiation of outer hair cells. J Cell Sci 119(Pt 14):2975-84. [PubMed: 16803873]  [MGI Ref ID J:111711]

Winter H; Ruttiger L; Muller M; Kuhn S; Brandt N; Zimmermann U; Hirt B; Bress A; Sausbier M; Conscience A; Flamant F; Tian Y; Zuo J; Pfister M; Ruth P; Lowenheim H; Samarut J; Engel J; Knipper M. 2009. Deafness in TRbeta mutants is caused by malformation of the tectorial membrane. J Neurosci 29(8):2581-7. [PubMed: 19244534]  [MGI Ref ID J:145941]

Wistuba J; Mittag J; Luetjens CM; Cooper TG; Yeung CH; Nieschlag E; Bauer K. 2007. Male congenital hypothyroid Pax8-/- mice are infertile despite adequate treatment with thyroid hormone. J Endocrinol 192(1):99-109. [PubMed: 17210747]  [MGI Ref ID J:117324]

Yu F; Gothe S; Wikstrom L; Forrest D; Vennstrom B; Larsson L. 2000. Effects of thyroid hormone receptor gene disruption on myosin isoform expression in mouse skeletal muscles. Am J Physiol Regul Integr Comp Physiol 278(6):R1545-54. [PubMed: 10848522]  [MGI Ref ID J:63071]

Zhang XY; Kaneshige M; Kamiya Y; Kaneshige K; McPhie P; Cheng SY. 2002. Differential expression of thyroid hormone receptor isoforms dictates the dominant negative activity of mutant Beta receptor. Mol Endocrinol 16(9):2077-92. [PubMed: 12198244]  [MGI Ref ID J:78703]

Zhu XG; Zhao L; Willingham MC; Cheng SY. 2010. Thyroid hormone receptors are tumor suppressors in a mouse model of metastatic follicular thyroid carcinoma. Oncogene 29(13):1909-19. [PubMed: 20062085]  [MGI Ref ID J:160390]

Health & husbandry

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

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryThis strain is maintained by mating homozygous siblings. Expected coat color from breeding:Black

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* $2525.00
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* $3283.00
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.

Control Information

  Control
   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.
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JAX® Mice
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JAX® Services
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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
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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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