Strain Name:

B6.Cg-Terctm1Rdp Dmdmdx-4Cv/BlauJ

Stock Number:

023535

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Availability:

Repository- Live

Use Restrictions Apply, see Terms of Use
Common Names: mdx/mTRKO;    
The mdx/mTRKO mouse model combines dystrophin-deficiency with telomere dysfunction/shortening, and may be a superior Duchenne muscular dystrophy model as it better recapitulates several of the human characteristics of DMD myopathology (progressive muscle weakness and damage, skeletal muscle fibrosis, diminished muscle stem cell regenerative capacity, dilated cardiomyopathy, heart failure and shortened life-span).

Description

Strain Information

Type Chemically Induced Mutation; Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating SystemSee Colony Maintenance under the Health & Care tab         (Female x Male)   04-APR-14
Specieslaboratory mouse
GenerationF?+pN1 (02-JUN-14)
Generation Definitions
 
Donating Investigator Helen M Blau,   Stanford University School of Medicine

Description
Duchenne muscular dystrophy (DMD) is a progressive muscular disorder caused by an imbalance between muscle degeneration and regeneration resulting in muscle degeneration, necrosis, accumulation of fat and fibrosis, and insufficient regeneration/loss of myofibers. The genetic cause of DMD are mutations of the dystrophin muscular dystrophy gene (DMD) on the X chromosome. Both the Dmdmdx (termination codon in exon 23) and Dmdmdx-4Cv (nonsense point mutation in exon 53) mutations in mice are predicted to express a truncated protein. Females heterozygous for either mutation are viable and fertile with no gross phenotypic abnormalities. Homozygous females and hemizygous males are viable and fertile with myopathic features of DMD; although the myopathology is both less severe than the human disease course and variable by mouse strain genetic background.
Specifically, the muscle pathology observed for C57BL/10ScSn-Dmdmdx mice (C57BL/10.mdx ; Stock No. 001801) and B6Ros.Cg-Dmdmdx-4Cv mice (B6.mdx-4Cv ; Stock No. 002378) includes active fiber necrosis, cellular infiltration, a wide range of fiber sizes, and numerous centrally nucleated regenerating fibers. However, despite the absence of dystrophin in skeletal and cardiac muscles, adult C57BL/10.mdx and B6.mdx-4Cv mice fail to exhibit several of features of DMD, including severe muscle weakness, progressive cardiomyopathy and shortened lifespan. In addition, these animals do not show other skeletal muscle characteristics of DMD (such as smaller number of myofibers, accumulation of fat and fibrosis, insufficient myofiber regeneration, and loss of muscle weight).
Differences between the two mutations exist. The Dmdmdx-4Cv allele has very low frequency of reversion to the wildtype allele in skeletal muscle (~10-fold lower frequency than the Dmdmdx mutation). In addition, the Dmdmdx-4Cv mutation affects more of the transcripts arising from alternative promoter usage within the dystrophin gene compared to the Dmdmdx mutation.

While telomere shortening is normally observed over time in mitotically active tissues, muscle tissue exhibits a lower proliferation rate and less telomere shortening with age. However, increased telomere shortening is associated with dystrophic human muscle cells and DMD patients. C57BL/6J mice homozygous for the telomerase RNA component null allele (C57BL/6J.mTR-/- ; Stock No. 004132) lack telomerase activity. Early generation homozygous mice have intact telomeres and appear grossly unaffected and healthy. However, telomere length is progressively shortened with successive generations of breeding mTR-/- mice together; resulting in dysfunction of the reproductive and hematopoietic systems, but little or no skeletal muscle abnormalities.


To investigate how telomere dysfunction affects the severity of muscular dystrophy seen in dystrophin-deficient mice, Dr. Helen M. Blau (Stanford University School of Medicine) created the double mutant mdx/mTR colony (Stock No. 023535) by breeding C57BL/6J-congenic mTR+/- mice with C57BL/6J-congenic Dmdmdx-4Cv mice. Males homozygous for the mTR null allele and hemizygous for the X-linked Dmdmdx-4Cv allele (mTR-/-;Dmdmdx-4Cv/Y), and females homozygous for both alleles (mTR-/-;Dmdmdx-4Cv/mdx-4Cv), are referred to as mdx/mTRKO. When compared to C57BL/10.mdx and B6.mdx-4Cv animals, the mdx/mTRKO mice exhibit additional features of severe human muscular dystrophy: including profound loss of muscle force, poor performance on a treadmill, increased serum creatine kinase levels, accumulation of fibrosis and calcium deposits within skeletal muscle tissues, kyphosis, dilated cardiomyopathy (ventricular dilation), cardiac contractile and conductance dysfunction, heart failure and shortened life-span. The severity of muscle wasting is concomitant with a decline in muscle stem cell regenerative capacity. mdx/mTRKO mice also exhibit telomere erosion (in cardiomyocytes, but not in other heart muscle cells), mitochondrial fragmentation and increased oxidative stress. The dystrophy phenotype becomes more severe with each successive generation of breeding mdx/mTRKO mice together (because such breeding results in progressively shorter telomere lengths with each generation). Specifically, mdx/mTRKO mice bred together for one generation (G1) exhibit cardiac dysfunction by ~80 weeks of age with death first occurring at ~30 weeks of age (~50% survival at 120 weeks of age). mdx/mTRKO G2 mice exhibit cardiac dysfunction by ~32 weeks of age with death first occurring at ~19 weeks of age (~50% survival at 80 weeks of age). mdx/mTRKO G3 mice exhibit cardiac dysfunction by ~8 weeks of age.

Development
These mdx/mTR mice harbor two mutations; the mTR null allele (Terctm1Rdp) and the X-linked muscular dystrophy mutation (Dmdmdx-4Cv).

The mTR null allele was designed by Dr. Ronald DePinho (Albert Einstein College of Medicine) with a neomycin cassette replacing the entire telomerase RNA component gene on chromosome 3. C57BL/6J-congenic mice harboring the mTR null allele are described and available from The Jackson Laboratory Repository as Stock No. 004132. Dr. Helen M. Blau (Stanford University School of Medicine) obtained heterozygous mTR mutant mice on a C57BL/6 genetic background from Dr. DePinho, and then bred them with C57BL/6J wildtype mice for six more generations. The resulting "C57BL6 mTRHet" mice (B6J.mTRHet) were used as described below.

The Dmdmdx-4Cv mutation was created in the laboratory of Dr. Verne M. Chapman (Roswell Park Memorial Institute) by N-ethyl-N-nitrosourea (ENU) treatment. Dmdmdx-4Cv has as a C-to-T transition (resulting in a termination codon) at position 7916 within exon 53 of the dystrophin muscular dystrophy gene (Dmd) on the X chromosome. B6Ros.Cg-Dmdmdx-4Cv mice are described and available from The Jackson Laboratory Repository as Stock No. 002378. Dr. Helen M. Blau (Stanford University School of Medicine) obtained Dmdmdx-4Cv mice on a C57BL/6 genetic background from Jeffrey S. Chamberlain (University of Washington), and then bred them with C57BL/6J wildtype mice for six generations. The resulting "C57BL6 mdx4cv" mice (B6J.mdx4cv) were used as described below.

To generate the double mutant line, Dr. Blau bred B6J.mTRHet mice with B6J.mdx4cv mice. Males heterozygous for the mTR null allele and hemizygous for the mdx4cv allele (called mdx4cv/mTRHet or mdx/mTRHet) were sent to The Jackson Laboratory Repository in 2013. Upon arrival, males were used to cryopreserve sperm. To establish our living mdx/mTR mouse colony, an aliquot of the frozen sperm was used to fertilize oocytes from C57BL/6J inbred females (Stock No. 000664).

Control Information

  Control
   See control note: Depending upon the nature of the experiment, the following mouse line(s) may be appropriate controls:
C57BL/6J.mTR-/- (Stock No. 004132: B6.Cg-Terctm1Rdp/J)
and/or B6.mdx-4Cv (Stock No. 002378: B6Ros.Cg-Dmdmdx-4Cv/J)
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Dmdmdx-4Cv allele
002378   B6Ros.Cg-Dmdmdx-4Cv/J
View Strains carrying   Dmdmdx-4Cv     (1 strain)

Strains carrying   Terctm1Rdp allele
004132   B6.Cg-Terctm1Rdp/J
018915   STOCK Terctm1Rdp Dmdmdx/J
View Strains carrying   Terctm1Rdp     (2 strains)

View Strains carrying other alleles of Dmd     (11 strains)

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).
Muscular Dystrophy, Duchenne Type; DMD
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Cardiomyopathy, Dilated, 3b; CMD3B   (DMD)
Dyskeratosis Congenita, Autosomal Dominant, 1; DKCA1   (TERC)
Muscular Dystrophy, Becker Type; BMD   (DMD)
Pulmonary Fibrosis and/or Bone Marrow Failure, Telomere-Related, 2;   (TERC)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Dmdmdx-4Cv/Dmdmdx-4Cv Terctm1Rdp/Terctm1Rdp

        B6.Cg-Terctm1Rdp Dmdmdx-4Cv
  • cardiovascular system phenotype
  • abnormal myocardial fiber morphology
    • cardiomyocyte nuclear area is moderately reduced in hearts of second generation (G2) 32 week old males as compared to controls   (MGI Ref ID J:200365)
    • cardiomyocyte diameter is smaller in hearts of 32 week old G2 males   (MGI Ref ID J:200365)
    • hearts from G2 males exhibit a loss of thick and thin myofilaments   (MGI Ref ID J:200365)
  • cardiac fibrosis
    • ventricular fibrosis is observed by 32 weeks in hearts of G2 males   (MGI Ref ID J:200365)
  • decreased ventricle muscle contractility
    • 32 week old G2 males exhibit reduced ventricular contractility as assessed by a reduction in fractional shortening as compared to controls   (MGI Ref ID J:200365)
  • dilated cardiomyopathy
    • reduced cardiac function is observed in older G1 males (80 weeks), but is not observed at 32 weeks   (MGI Ref ID J:200365)
    • cardiac dysfunction is observed at 8 weeks in third generation males   (MGI Ref ID J:200365)
    • cardiac dysfunction can be induced by angiotension II infusion in younger second generation males   (MGI Ref ID J:200365)
  • dilated heart left ventricle
    • increased chamber size is observed in 32 week old G2 males as compared to controls   (MGI Ref ID J:200365)
    • left ventricular transverse area is increased in diastole and systole in G2 males   (MGI Ref ID J:200365)
  • increased heart left ventricle size
    • left ventricle enlargement is observed by 32 weeks in hearts of G2 males as compared to controls   (MGI Ref ID J:200365)
  • prolonged QRS complex duration
    • wide QRS interval is observed in both G1 and G2 males as compared to controls   (MGI Ref ID J:200365)
  • ventricular cardiomyopathy   (MGI Ref ID J:200365)
  • cellular phenotype
  • abnormal mitochondrial shape
    • extensive mitochondrial fragmentation observed in cardiac muscle of second generation mice   (MGI Ref ID J:200365)
  • abnormal telomere length
    • 50% reduction in telomere length in G2 cardiomyocytes as compared to controls   (MGI Ref ID J:200365)
    • telomere lengths are similar to controls in vascular smooth muscle cells   (MGI Ref ID J:200365)
  • decreased mitochondria size
    • mitochondria size is decreaseed in cardiac muscle of second generation mice   (MGI Ref ID J:200365)
  • disorganized mitochondrial cristae
    • lack of well-defined cristae observed in cardiac muscle of second generation mice   (MGI Ref ID J:200365)
  • increased mitochondria number
    • moderately increased number of mitochondria is observed in cardiac muscle of second generation mice   (MGI Ref ID J:200365)
  • oxidative stress
    • an increased number of 8-OHdg-positive nuclei, a marker of oxidative damage, is observed in G2 hearts as compared to controls   (MGI Ref ID J:200365)
  • mortality/aging
  • premature death
    • death occurs as early as 30 weeks in first generation males, T50 is 120 weeks   (MGI Ref ID J:200365)
    • death occurs as early as 19 weeks in second generation males, T50 is 80 weeks   (MGI Ref ID J:200365)
  • muscle phenotype
  • abnormal myocardial fiber morphology
    • cardiomyocyte nuclear area is moderately reduced in hearts of second generation (G2) 32 week old males as compared to controls   (MGI Ref ID J:200365)
    • cardiomyocyte diameter is smaller in hearts of 32 week old G2 males   (MGI Ref ID J:200365)
    • hearts from G2 males exhibit a loss of thick and thin myofilaments   (MGI Ref ID J:200365)
  • decreased ventricle muscle contractility
    • 32 week old G2 males exhibit reduced ventricular contractility as assessed by a reduction in fractional shortening as compared to controls   (MGI Ref ID J:200365)
  • dilated cardiomyopathy
    • reduced cardiac function is observed in older G1 males (80 weeks), but is not observed at 32 weeks   (MGI Ref ID J:200365)
    • cardiac dysfunction is observed at 8 weeks in third generation males   (MGI Ref ID J:200365)
    • cardiac dysfunction can be induced by angiotension II infusion in younger second generation males   (MGI Ref ID J:200365)
  • ventricular cardiomyopathy   (MGI Ref ID J:200365)
  • homeostasis/metabolism phenotype
  • cardiac fibrosis
    • ventricular fibrosis is observed by 32 weeks in hearts of G2 males   (MGI Ref ID J:200365)
View Research Applications

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

Cancer Research
Genes Regulating Growth and Proliferation
Other
      DNA Repair
Tumor Suppressor Genes

Cardiovascular Research
Heart Abnormalities
      cardiomyopathy
Other

Cell Biology Research
Channel and Transporter Defects
DNA Damage Response
Protein Processing
Signal Transduction

Developmental Biology Research
Internal/Organ Defects
      heart
      hematopoietic defects
Lymphoid Tissue Defects
      hematopoietic defects
Mesodermal Defects
      Myogenesis Defects

Hematological Research
Hematopoietic Defects
Immunological Defects

Internal/Organ Research
Heart Abnormalities

Neurobiology Research
Ataxia (Movement) Defects
Channel and Transporter Defects
Muscular Dystrophy
      Duchenne type

Reproductive Biology Research
Developmental Defects Affecting Gonads
      germ cell deficient

Research Tools
Cardiovascular Research
Cell Biology Research
Genetics Research
Hematological Research
Reproductive Biology Research

Dmdmdx-4Cv related

Neurobiology Research
Muscular Dystrophy
      Duchenne type

Terctm1Rdp related

Cancer Research
Genes Regulating Growth and Proliferation
Other
      DNA Repair
Tumor Suppressor Genes

Cell Biology Research
DNA Damage Response

Research Tools
Cell Biology Research
Genetics Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Dmdmdx-4Cv
Allele Name X linked muscular dystrophy 4, Verne Chapman
Allele Type Chemically induced (ENU)
Common Name(s) mdx4cv; mdx4cv; mdx4cv; mdxCv4;
Mutation Made ByDr. Verne Chapman (deceased),   Roswell Park Memorial Institute
Strain of OriginC3Ha.Cg-Hprt Pgk1
Gene Symbol and Name Dmd, dystrophin, muscular dystrophy
Chromosome X
Gene Common Name(s) BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dp427; Dp71; Duchenne muscular dystrophy; MRX85; X-linked muscular dystrophy; dys; mdx; pke; pyruvate kinase expression;
Molecular Note A C to T transition in exon 53 at position 7916 creates a premature stop codon. [MGI Ref ID J:34517]
 
Allele Symbol Terctm1Rdp
Allele Name targeted mutation 1, Ronald DePinho
Allele Type Targeted (Null/Knockout)
Common Name(s) TR-; Terc-; mTR-; mTerc-;
Mutation Made ByDr. Carol Greider,   Johns Hopkins Univ School of Medicine
Strain of OriginSTOCK 129/Sv and C57BL/6J and SJL
ES Cell Line NameWW6
ES Cell Line StrainSTOCK 129/Sv and C57BL/6J and SJL
Gene Symbol and Name Terc, telomerase RNA component
Chromosome 3
Gene Common Name(s) mTER; mTR;
Molecular Note Replacement of the entire gene with a neomycin cassette. [MGI Ref ID J:43517]

Genotyping

Genotyping Information

Genotyping Protocols

Dmdmdx-4Cv, Pyrosequencing
Terctm1Rdpalternate2,

MELT



Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Mourkioti F; Kustan J; Kraft P; Day JW; Zhao MM; Kost-Alimova M; Protopopov A; DePinho RA; Bernstein D; Meeker AK; Blau HM. 2013. Role of telomere dysfunction in cardiac failure in Duchenne muscular dystrophy. Nat Cell Biol 15(8):895-904. [PubMed: 23831727]  [MGI Ref ID J:200365]

Sacco A; Mourkioti F; Tran R; Choi J; Llewellyn M; Kraft P; Shkreli M; Delp S; Pomerantz JH; Artandi SE; Blau HM. 2010. Short telomeres and stem cell exhaustion model Duchenne muscular dystrophy in mdx/mTR mice. Cell 143(7):1059-71. [PubMed: 21145579]  [MGI Ref ID J:167294]

Additional References

Dmdmdx-4Cv related

Adamo CM; Dai DF; Percival JM; Minami E; Willis MS; Patrucco E; Froehner SC; Beavo JA. 2010. Sildenafil reverses cardiac dysfunction in the mdx mouse model of Duchenne muscular dystrophy. Proc Natl Acad Sci U S A 107(44):19079-83. [PubMed: 20956307]  [MGI Ref ID J:166234]

Aigner B; Rathkolb B; Klaften M; Sedlmeier R; Klempt M; Wagner S; Michel D; Mayer U; Klopstock T; de Angelis MH; Wolf E. 2009. Generation of N-ethyl-N-nitrosourea-induced mouse mutants with deviations in plasma enzyme activities as novel organ-specific disease models. Exp Physiol 94(4):412-21. [PubMed: 19151073]  [MGI Ref ID J:187875]

Arpke RW; Darabi R; Mader TL; Zhang Y; Toyama A; Lonetree CL; Nash N; Lowe DA; Perlingeiro RC; Kyba M. 2013. A New Immuno- Dystrophin-Deficient Model, the NSG-Mdx Mouse, Provides Evidence for Functional Improvement Following Allogeneic Satellite Cell Transplantation. Stem Cells :. [PubMed: 23606600]  [MGI Ref ID J:199729]

Banks GB; Combs AC; Chamberlain JR; Chamberlain JS. 2008. Molecular and cellular adaptations to chronic myotendinous strain injury in mdx mice expressing a truncated dystrophin. Hum Mol Genet 17(24):3975-86. [PubMed: 18799475]  [MGI Ref ID J:142565]

Chapman VM; Miller DR; Armstrong D; Caskey CT. 1989. Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice. Proc Natl Acad Sci U S A 86(4):1292-6. [PubMed: 2919177]  [MGI Ref ID J:9638]

Chretien F; Dreyfus PA; Christov C; Caramelle P; Lagrange JL; Chazaud B; Gherardi RK. 2005. In vivo fusion of circulating fluorescent cells with dystrophin-deficient myofibers results in extensive sarcoplasmic fluorescence expression but limited dystrophin sarcolemmal expression. Am J Pathol 166(6):1741-8. [PubMed: 15920159]  [MGI Ref ID J:98788]

Claeys KG; Sozanska M; Martin JJ; Lacene E; Vignaud L; Stockholm D; Laforet P; Eymard B; Kichler A; Scherman D; Voit T; Israeli D. 2010. DNAJB2 expression in normal and diseased human and mouse skeletal muscle. Am J Pathol 176(6):2901-10. [PubMed: 20395441]  [MGI Ref ID J:161160]

Cordani N; Pisa V; Pozzi L; Sciorati C; Clementi E. 2014. Nitric oxide controls fat deposition in dystrophic skeletal muscle by regulating fibro-adipogenic precursor differentiation. Stem Cells 32(4):874-85. [PubMed: 24170326]  [MGI Ref ID J:210190]

Danko I; Chapman V; Wolff JA. 1992. The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr Res 32(1):128-31. [PubMed: 1635838]  [MGI Ref ID J:23502]

Gayraud-Morel B; Chretien F; Flamant P; Gomes D; Zammit PS; Tajbakhsh S. 2007. A role for the myogenic determination gene Myf5 in adult regenerative myogenesis. Dev Biol 312(1):13-28. [PubMed: 17961534]  [MGI Ref ID J:128921]

Howard PL; Dally GY; Wong MH; Ho A; Weleber RG; Pillers DA; Ray PN. 1998. Localization of dystrophin isoform Dp71 to the inner limiting membrane of the retina suggests a unique functional contribution of Dp71 in the retina. Hum Mol Genet 7(9):1385-91. [PubMed: 9700191]  [MGI Ref ID J:115134]

Im WB; Phelps SF; Copen EH; Adams EG; Slightom JL; Chamberlain JS. 1996. Differential expression of dystrophin isoforms in strains of mdx mice with different mutations. Hum Mol Genet 5(8):1149-53. [PubMed: 8842734]  [MGI Ref ID J:34517]

Judge LM; Haraguchiln M; Chamberlain JS. 2006. Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex. J Cell Sci 119(Pt 8):1537-46. [PubMed: 16569668]  [MGI Ref ID J:107808]

Khouzami L; Bourin MC; Christov C; Damy T; Escoubet B; Caramelle P; Perier M; Wahbi K; Meune C; Pavoine C; Pecker F. 2010. Delayed cardiomyopathy in dystrophin deficient mdx mice relies on intrinsic glutathione resource. Am J Pathol 177(3):1356-64. [PubMed: 20696779]  [MGI Ref ID J:163690]

Laure L; Suel L; Roudaut C; Bourg N; Ouali A; Bartoli M; Richard I; Daniele N. 2009. Cardiac ankyrin repeat protein is a marker of skeletal muscle pathological remodelling. FEBS J 276(3):669-84. [PubMed: 19143834]  [MGI Ref ID J:147891]

Li D; Shin JH; Duan D. 2011. iNOS Ablation Does Not Improve Specific Force of the Extensor Digitorum Longus Muscle in Dystrophin-Deficient mdx4cv Mice. PLoS One 6(6):e21618. [PubMed: 21738735]  [MGI Ref ID J:174763]

Li D; Yue Y; Duan D. 2008. Preservation of muscle force in mdx3cv mice correlates with low-level expression of a near full-length dystrophin protein. Am J Pathol 172(5):1332-41. [PubMed: 18385524]  [MGI Ref ID J:134272]

Li D; Yue Y; Lai Y; Hakim CH; Duan D. 2011. Nitrosative stress elicited by nNOSmicro delocalization inhibits muscle force in dystrophin-null mice. J Pathol 223(1):88-98. [PubMed: 21125668]  [MGI Ref ID J:167308]

Li S; Kimura E; Ng R; Fall BM; Meuse L; Reyes M; Faulkner JA; Chamberlain JS. 2006. A highly functional mini-dystrophin/GFP fusion gene for cell and gene therapy studies of Duchenne muscular dystrophy. Hum Mol Genet 15(10):1610-22. [PubMed: 16595609]  [MGI Ref ID J:144130]

Nguyen-Tran DH; Hait NC; Sperber H; Qi J; Fischer K; Ieronimakis N; Pantoja M; Hays A; Allegood J; Reyes M; Spiegel S; Ruohola-Baker H. 2014. Molecular mechanism of sphingosine-1-phosphate action in Duchenne muscular dystrophy. Dis Model Mech 7(1):41-54. [PubMed: 24077965]  [MGI Ref ID J:208735]

Pillers DA; Weleber RG; Green DG; Rash SM; Dally GY; Howard PL ; Powers MR ; Hood DC ; Chapman VM ; Ray PN ; Woodward WR. 1999. Effects of dystrophin isoforms on signal transduction through neural retina: genotype-phenotype analysis of duchenne muscular dystrophy mouse mutants. Mol Genet Metab 66(2):100-10. [PubMed: 10068512]  [MGI Ref ID J:53822]

Shin JH; Hakim CH; Zhang K; Duan D. 2011. Genotyping mdx, mdx3cv, and mdx4cv mice by primer competition polymerase chain reaction. Muscle Nerve 43(2):283-6. [PubMed: 21254096]  [MGI Ref ID J:169288]

Tanaka KK; Hall JK; Troy AA; Cornelison DD; Majka SM; Olwin BB. 2009. Syndecan-4-expressing muscle progenitor cells in the SP engraft as satellite cells during muscle regeneration. Cell Stem Cell 4(3):217-25. [PubMed: 19265661]  [MGI Ref ID J:149919]

Vignier N; Amor F; Fogel P; Duvallet A; Poupiot J; Charrier S; Arock M; Montus M; Nelson I; Richard I; Carrier L; Servais L; Voit T; Bonne G; Israeli D. 2013. Distinctive serum miRNA profile in mouse models of striated muscular pathologies. PLoS One 8(2):e55281. [PubMed: 23418438]  [MGI Ref ID J:199417]

Terctm1Rdp related

Akbay EA; Contreras CM; Perera SA; Sullivan JP; Broaddus RR; Schorge JO; Ashfaq R; Saboorian H; Wong KK; Castrillon DH. 2008. Differential roles of telomere attrition in type I and II endometrial carcinogenesis. Am J Pathol 173(2):536-44. [PubMed: 18599611]  [MGI Ref ID J:138286]

Allsopp RC; Morin GB; DePinho R; Harley CB; Weissman IL. 2003. Telomerase is required to slow telomere shortening and extend replicative lifespan of HSCs during serial transplantation. Blood 102(2):517-20. [PubMed: 12663456]  [MGI Ref ID J:115695]

Argilla D; Chin K; Singh M; Hodgson JG; Bosenberg M; de Solorzano CO; Lockett S; DePinho RA; Gray J; Hanahan D. 2004. Absence of telomerase and shortened telomeres have minimal effects on skin and pancreatic carcinogenesis elicited by viral oncogenes. Cancer Cell 6(4):373-85. [PubMed: 15488760]  [MGI Ref ID J:94774]

Armanios M; Alder JK; Parry EM; Karim B; Strong MA; Greider CW. 2009. Short telomeres are sufficient to cause the degenerative defects associated with aging. Am J Hum Genet 85(6):823-32. [PubMed: 19944403]  [MGI Ref ID J:158466]

Artandi SE; Chang S; Lee SL; Alson S; Gottlieb GJ; Chin L; DePinho RA. 2000. Telomere dysfunction promotes non-reciprocal translocations and epithelial cancers in mice [see comments] Nature 406(6796):641-5. [PubMed: 10949306]  [MGI Ref ID J:63843]

Autexier C. 2008. POT of gold: modeling dyskeratosis congenita in the mouse. Genes Dev 22(13):1731-6. [PubMed: 18593874]  [MGI Ref ID J:137424]

Begus-Nahrmann Y; Hartmann D; Kraus J; Eshraghi P; Scheffold A; Grieb M; Rasche V; Schirmacher P; Lee HW; Kestler HA; Lechel A; Rudolph KL. 2012. Transient telomere dysfunction induces chromosomal instability and promotes carcinogenesis. J Clin Invest 122(6):2283-8. [PubMed: 22622037]  [MGI Ref ID J:190483]

Begus-Nahrmann Y; Lechel A; Obenauf AC; Nalapareddy K; Peit E; Hoffmann E; Schlaudraff F; Liss B; Schirmacher P; Kestler H; Danenberg E; Barker N; Clevers H; Speicher MR; Rudolph KL. 2009. p53 deletion impairs clearance of chromosomal-instable stem cells in aging telomere-dysfunctional mice. Nat Genet 41(10):1138-43. [PubMed: 19718028]  [MGI Ref ID J:155011]

Benetti R; Garcia-Cao M; Blasco MA. 2007. Telomere length regulates the epigenetic status of mammalian telomeres and subtelomeres. Nat Genet 39(2):243-50. [PubMed: 17237781]  [MGI Ref ID J:118330]

Bhattacharjee RN; Banerjee B; Akira S; Hande MP. 2010. Telomere-mediated chromosomal instability triggers TLR4 induced inflammation and death in mice. PLoS One 5(7):e11873. [PubMed: 20686699]  [MGI Ref ID J:163068]

Blanco R; Munoz P; Flores JM; Klatt P; Blasco MA. 2007. Telomerase abrogation dramatically accelerates TRF2-induced epithelial carcinogenesis. Genes Dev 21(2):206-20. [PubMed: 17234886]  [MGI Ref ID J:117418]

Blasco MA; Lee HW; Hande MP; Samper E; Lansdorp PM; DePinho RA ; Greider CW. 1997. Telomere shortening and tumor formation by mouse cells lacking telomerase RNA [see comments] Cell 91(1):25-34. [PubMed: 9335332]  [MGI Ref ID J:43517]

Bojovic B; Crowe DL. 2011. Telomere dysfunction promotes metastasis in a TERC null mouse model of head and neck cancer. Mol Cancer Res 9(7):901-13. [PubMed: 21593138]  [MGI Ref ID J:205225]

Bu DX; Johansson ME; Ren J; Xu DW; Johnson FB; Edfeldt K; Yan ZQ. 2010. Nuclear factor {kappa}B-mediated transactivation of telomerase prevents intimal smooth muscle cell from replicative senescence during vascular repair. Arterioscler Thromb Vasc Biol 30(12):2604-10. [PubMed: 20864668]  [MGI Ref ID J:183205]

Cayuela ML; Flores JM; Blasco MA. 2005. The telomerase RNA component Terc is required for the tumour-promoting effects of Tert overexpression. EMBO Rep 6(3):268-74. [PubMed: 15731767]  [MGI Ref ID J:96945]

Chamberlain JS. 2010. Duchenne muscular dystrophy models show their age. Cell 143(7):1040-2. [PubMed: 21183068]  [MGI Ref ID J:167707]

Chang S. 2005. Modeling aging and cancer in the telomerase knockout mouse. Mutat Res 576(1-2):39-53. [PubMed: 15927211]  [MGI Ref ID J:99987]

Chang S; Multani AS; Cabrera NG; Naylor ML; Laud P; Lombard D; Pathak S; Guarente L; DePinho RA. 2004. Essential role of limiting telomeres in the pathogenesis of Werner syndrome. Nat Genet 36(8):877-82. [PubMed: 15235603]  [MGI Ref ID J:91715]

Chiang YJ; Hemann MT; Hathcock KS; Tessarollo L; Feigenbaum L; Hahn WC; Hodes RJ. 2004. Expression of telomerase RNA template, but not telomerase reverse transcriptase, is limiting for telomere length maintenance in vivo. Mol Cell Biol 24(16):7024-31. [PubMed: 15282303]  [MGI Ref ID J:92243]

Chin L; Artandi SE; Shen Q; Tam A; Lee SL; Gottlieb GJ; Greider CW; DePinho RA. 1999. p53 deficiency rescues the adverse effects of telomere loss and cooperates with telomere dysfunction to accelerate carcinogenesis. Cell 97(4):527-38. [PubMed: 10338216]  [MGI Ref ID J:54988]

Choudhury AR; Ju Z; Djojosubroto MW; Schienke A; Lechel A; Schaetzlein S; Jiang H; Stepczynska A; Wang C; Buer J; Lee HW; von Zglinicki T; Ganser A; Schirmacher P; Nakauchi H; Rudolph KL. 2007. Cdkn1a deletion improves stem cell function and lifespan of mice with dysfunctional telomeres without accelerating cancer formation. Nat Genet 39(1):99-105. [PubMed: 17143283]  [MGI Ref ID J:117492]

Cosme-Blanco W; Shen MF; Lazar AJ; Pathak S; Lozano G; Multani AS; Chang S. 2007. Telomere dysfunction suppresses spontaneous tumorigenesis in vivo by initiating p53-dependent cellular senescence. EMBO Rep 8(5):497-503. [PubMed: 17396137]  [MGI Ref ID J:208249]

Denchi EL. 2009. Give me a break: How telomeres suppress the DNA damage response. DNA Repair (Amst) 8(9):1118-26. [PubMed: 19482563]  [MGI Ref ID J:151432]

Du X; Shen J; Kugan N; Furth EE; Lombard DB; Cheung C; Pak S; Luo G; Pignolo RJ; DePinho RA; Guarente L; Johnson FB. 2004. Telomere shortening exposes functions for the mouse werner and bloom syndrome genes. Mol Cell Biol 24(19):8437-46. [PubMed: 15367665]  [MGI Ref ID J:93016]

Espejel S; Franco S; Rodriguez-Perales S; Bouffler SD; Cigudosa JC; Blasco MA. 2002. Mammalian Ku86 mediates chromosomal fusions and apoptosis caused by critically short telomeres. EMBO J 21(9):2207-19. [PubMed: 11980718]  [MGI Ref ID J:76498]

Espejel S; Franco S; Sgura A; Gae D; Bailey SM; Taccioli GE; Blasco MA. 2002. Functional interaction between DNA-PKcs and telomerase in telomere length maintenance. EMBO J 21(22):6275-87. [PubMed: 12426399]  [MGI Ref ID J:111398]

Espejel S; Klatt P; Menissier-de Murcia J; Martin-Caballero J; Flores JM; Taccioli G; de Murcia G; Blasco MA. 2004. Impact of telomerase ablation on organismal viability, aging, and tumorigenesis in mice lacking the DNA repair proteins PARP-1, Ku86, or DNA-PKcs. J Cell Biol 167(4):627-38. [PubMed: 15545322]  [MGI Ref ID J:94117]

Farazi PA; Glickman J; Jiang S; Yu A; Rudolph KL; DePinho RA. 2003. Differential impact of telomere dysfunction on initiation and progression of hepatocellular carcinoma. Cancer Res 63(16):5021-7. [PubMed: 12941829]  [MGI Ref ID J:85141]

Feldser D; Strong MA; Greider CW. 2006. Ataxia telangiectasia mutated (Atm) is not required for telomerase-mediated elongation of short telomeres. Proc Natl Acad Sci U S A 103(7):2249-51. [PubMed: 16467146]  [MGI Ref ID J:106070]

Feldser DM; Greider CW. 2007. Short telomeres limit tumor progression in vivo by inducing senescence. Cancer Cell 11(5):461-9. [PubMed: 17433785]  [MGI Ref ID J:121351]

Ferron S; Mira H; Franco S; Cano-Jaimez M; Bellmunt E; Ramirez C; Farinas I; Blasco MA. 2004. Telomere shortening and chromosomal instability abrogates proliferation of adult but not embryonic neural stem cells. Development 131(16):4059-70. [PubMed: 15269166]  [MGI Ref ID J:92059]

Ferron SR; Marques-Torrejon MA; Mira H; Flores I; Taylor K; Blasco MA; Farinas I. 2009. Telomere shortening in neural stem cells disrupts neuronal differentiation and neuritogenesis. J Neurosci 29(46):14394-407. [PubMed: 19923274]  [MGI Ref ID J:158281]

Flores I; Blasco MA. 2009. A p53-dependent response limits epidermal stem cell functionality and organismal size in mice with short telomeres. PLoS ONE 4(3):e4934. [PubMed: 19295915]  [MGI Ref ID J:147468]

Flores I; Canela A; Vera E; Tejera A; Cotsarelis G; Blasco MA. 2008. The longest telomeres: a general signature of adult stem cell compartments. Genes Dev 22(5):654-67. [PubMed: 18283121]  [MGI Ref ID J:131719]

Flores I; Cayuela ML; Blasco MA. 2005. Effects of telomerase and telomere length on epidermal stem cell behavior. Science 309(5738):1253-6. [PubMed: 16037417]  [MGI Ref ID J:100470]

Flores I; Evan G; Blasco MA. 2006. Genetic analysis of myc and telomerase interactions in vivo. Mol Cell Biol 26(16):6130-8. [PubMed: 16880523]  [MGI Ref ID J:111405]

Franco S; Alsheimer M; Herrera E; Benavente R; Blasco MA. 2002. Mammalian meiotic telomeres: composition and ultrastructure in telomerase-deficient mice. Eur J Cell Biol 81(6):335-40. [PubMed: 12113474]  [MGI Ref ID J:102812]

Franco S; Canela A; Klatt P; Blasco MA. 2005. Effectors of mammalian telomere dysfunction: a comparative transcriptome analysis using mouse models. Carcinogenesis 26(9):1613-26. [PubMed: 15860505]  [MGI Ref ID J:100743]

Franco S; Segura I; Riese HH; Blasco MA. 2002. Decreased B16F10 melanoma growth and impaired vascularization in telomerase-deficient mice with critically short telomeres. Cancer Res 62(2):552-9. [PubMed: 11809709]  [MGI Ref ID J:74001]

Franco S; van de Vrugt HJ; Fernandez P; Aracil M; Arwert F; Blasco MA. 2004. Telomere dynamics in Fancg-deficient mouse and human cells. Blood 104(13):3927-35. [PubMed: 15319283]  [MGI Ref ID J:95292]

Frescas D; de Lange T. 2014. A TIN2 dyskeratosis congenita mutation causes telomerase-independent telomere shortening in mice. Genes Dev 28(2):153-66. [PubMed: 24449270]  [MGI Ref ID J:207367]

Garcia-Cao I; Garcia-Cao M; Tomas-Loba A; Martin-Caballero J; Flores JM; Klatt P; Blasco MA; Serrano M. 2006. Increased p53 activity does not accelerate telomere-driven ageing. EMBO Rep 7(5):546-52. [PubMed: 16582880]  [MGI Ref ID J:116879]

Ghosh A; Saginc G; Leow SC; Khattar E; Shin EM; Yan TD; Wong M; Zhang Z; Li G; Sung WK; Zhou J; Chng WJ; Li S; Liu E; Tergaonkar V. 2012. Telomerase directly regulates NF-kappaB-dependent transcription. Nat Cell Biol 14(12):1270-81. [PubMed: 23159929]  [MGI Ref ID J:195250]

Gonzalez-Suarez E; Goytisolo FA; Flores JM; Blasco MA. 2003. Telomere dysfunction results in enhanced organismal sensitivity to the alkylating agent N-methyl-N-nitrosourea. Cancer Res 63(21):7047-50. [PubMed: 14612493]  [MGI Ref ID J:87029]

Gonzalez-Suarez E; Samper E; Flores JM; Blasco MA. 2000. Telomerase-deficient mice with short telomeres are resistant to skin tumorigenesis Nat Genet 26(1):114-7. [PubMed: 10973262]  [MGI Ref ID J:64363]

Greenberg RA; Chin L; Femino A; Lee KH; Gottlieb GJ; Singer RH; Greider CW; DePinho RA. 1999. Short dysfunctional telomeres impair tumorigenesis in the INK4a(delta2/3) cancer-prone mouse. Cell 97(4):515-25. [PubMed: 10338215]  [MGI Ref ID J:54989]

Gu BW; Bessler M; Mason PJ. 2008. A pathogenic dyskerin mutation impairs proliferation and activates a DNA damage response independent of telomere length in mice. Proc Natl Acad Sci U S A 105(29):10173-8. [PubMed: 18626023]  [MGI Ref ID J:138335]

Guo N; Parry EM; Li LS; Kembou F; Lauder N; Hussain MA; Berggren PO; Armanios M. 2011. Short telomeres compromise beta-cell signaling and survival. PLoS One 6(3):e17858. [PubMed: 21423765]  [MGI Ref ID J:171692]

Hande MP; Samper E; Lansdorp P; Blasco MA. 1999. Telomere length dynamics and chromosomal instability in cells derived from telomerase null mice. J Cell Biol 144(4):589-601. [PubMed: 10037783]  [MGI Ref ID J:53241]

Hao LY; Armanios M; Strong MA; Karim B; Feldser DM; Huso D; Greider CW. 2005. Short telomeres, even in the presence of telomerase, limit tissue renewal capacity. Cell 123(6):1121-31. [PubMed: 16360040]  [MGI Ref ID J:115778]

Hao LY; Greider CW. 2004. Genomic instability in both wild-type and telomerase null MEFs. Chromosoma 113(2):62-8. [PubMed: 15258806]  [MGI Ref ID J:103501]

Hao LY; Strong MA; Greider CW. 2004. Phosphorylation of H2AX at short telomeres in T cells and fibroblasts. J Biol Chem 279(43):45148-54. [PubMed: 15322096]  [MGI Ref ID J:93974]

Hathcock KS; Hemann MT; Opperman KK; Strong MA; Greider CW; Hodes RJ. 2002. Haploinsufficiency of mTR results in defects in telomere elongation. Proc Natl Acad Sci U S A 99(6):3591-6. [PubMed: 11904421]  [MGI Ref ID J:81782]

He H; Wang Y; Guo X; Ramchandani S; Ma J; Shen MF; Garcia DA; Deng Y; Multani AS; You MJ; Chang S. 2009. Pot1b deletion and telomerase haploinsufficiency in mice initiate an ATR-dependent DNA damage response and elicit phenotypes resembling dyskeratosis congenita. Mol Cell Biol 29(1):229-40. [PubMed: 18936156]  [MGI Ref ID J:144553]

Hemann MT; Greider CW. 1999. G-strand overhangs on telomeres in telomerase-deficient mouse cells. Nucleic Acids Res 27(20):3964-9. [PubMed: 10497259]  [MGI Ref ID J:58129]

Hemann MT; Strong MA; Hao LY; Greider CW. 2001. The shortest telomere, not average telomere length, is critical for cell viability and chromosome stability. Cell 107(1):67-77. [PubMed: 11595186]  [MGI Ref ID J:107711]

Herrera E; Martinez-A C; Blasco MA. 2000. Impaired germinal center reaction in mice with short telomeres. EMBO J 19(3):472-81. [PubMed: 10654945]  [MGI Ref ID J:60223]

Herrera E; Samper E; Blasco MA. 1999. Telomere shortening in mTR-/- embryos is associated with failure to close the neural tube. EMBO J 18(5):1172-81. [PubMed: 10064584]  [MGI Ref ID J:53600]

Herrera E; Samper E; Martin-Caballero J; Flores JM; Lee HW; Blasco MA. 1999. Disease states associated with telomerase deficiency appear earlier in mice with short telomeres. EMBO J 18(11):2950-60. [PubMed: 10357808]  [MGI Ref ID J:55545]

Hockemeyer D; Daniels JP; Takai H; de Lange T. 2006. Recent expansion of the telomeric complex in rodents: Two distinct POT1 proteins protect mouse telomeres. Cell 126(1):63-77. [PubMed: 16839877]  [MGI Ref ID J:112185]

Hockemeyer D; Palm W; Wang RC; Couto SS; de Lange T. 2008. Engineered telomere degradation models dyskeratosis congenita. Genes Dev 22(13):1773-85. [PubMed: 18550783]  [MGI Ref ID J:137372]

Hu J; Hwang SS; Liesa M; Gan B; Sahin E; Jaskelioff M; Ding Z; Ying H; Boutin AT; Zhang H; Johnson S; Ivanova E; Kost-Alimova M; Protopopov A; Wang YA; Shirihai OS; Chin L; Depinho RA. 2012. Antitelomerase Therapy Provokes ALT and Mitochondrial Adaptive Mechanisms in Cancer. Cell 148(4):651-63. [PubMed: 22341440]  [MGI Ref ID J:181456]

Jackson SR; Lee J; Reddy R; Williams GN; Kikuchi A; Freiberg Y; Warburton D; Driscoll B. 2011. Partial pneumonectomy of telomerase null mice carrying shortened telomeres initiates cell growth arrest resulting in a limited compensatory growth response. Am J Physiol Lung Cell Mol Physiol 300(6):L898-909. [PubMed: 21460122]  [MGI Ref ID J:174244]

Jiang H; Schiffer E; Song Z; Wang J; Zurbig P; Thedieck K; Moes S; Bantel H; Saal N; Jantos J; Brecht M; Jeno P; Hall MN; Hager K; Manns MP; Hecker H; Ganser A; Dohner K; Bartke A; Meissner C; Mischak H; Ju Z; Rudolph KL. 2008. Proteins induced by telomere dysfunction and DNA damage represent biomarkers of human aging and disease. Proc Natl Acad Sci U S A 105(32):11299-304. [PubMed: 18695223]  [MGI Ref ID J:140477]

Ju Z; Jiang H; Jaworski M; Rathinam C; Gompf A; Klein C; Trumpp A; Rudolph KL. 2007. Telomere dysfunction induces environmental alterations limiting hematopoietic stem cell function and engraftment. Nat Med 13(6):742-7. [PubMed: 17486088]  [MGI Ref ID J:121891]

Karlseder J; Kachatrian L; Takai H; Mercer K; Hingorani S; Jacks T; de Lange T. 2003. Targeted deletion reveals an essential function for the telomere length regulator Trf1. Mol Cell Biol 23(18):6533-41. [PubMed: 12944479]  [MGI Ref ID J:85440]

Khoo CM; Carrasco DR; Bosenberg MW; Paik JH; Depinho RA. 2007. Ink4a/Arf tumor suppressor does not modulate the degenerative conditions or tumor spectrum of the telomerase-deficient mouse. Proc Natl Acad Sci U S A 104(10):3931-6. [PubMed: 17360455]  [MGI Ref ID J:120065]

Laud PR; Multani AS; Bailey SM; Wu L; Ma J; Kingsley C; Lebel M; Pathak S; DePinho RA; Chang S. 2005. Elevated telomere-telomere recombination in WRN-deficient, telomere dysfunctional cells promotes escape from senescence and engagement of the ALT pathway. Genes Dev 19(21):2560-70. [PubMed: 16264192]  [MGI Ref ID J:102524]

Le R; Kou Z; Jiang Y; Li M; Huang B; Liu W; Li H; Kou X; He W; Rudolph KL; Ju Z; Gao S. 2014. Enhanced telomere rejuvenation in pluripotent cells reprogrammed via nuclear transfer relative to induced pluripotent stem cells. Cell Stem Cell 14(1):27-39. [PubMed: 24268696]  [MGI Ref ID J:205597]

Lechel A; Holstege H; Begus Y; Schienke A; Kamino K; Lehmann U; Kubicka S; Schirmacher P; Jonkers J; Rudolph KL. 2007. Telomerase deletion limits progression of p53-mutant hepatocellular carcinoma with short telomeres in chronic liver disease. Gastroenterology 132(4):1465-75. [PubMed: 17433324]  [MGI Ref ID J:128326]

Lee HW; Blasco MA; Gottlieb GJ; Horner JW 2nd; Greider CW; DePinho RA. 1998. Essential role of mouse telomerase in highly proliferative organs. Nature 392(6676):569-74. [PubMed: 9560153]  [MGI Ref ID J:46933]

Lee J; Reddy R; Barsky L; Scholes J; Chen H; Shi W; Driscoll B. 2009. Lung alveolar integrity is compromised by telomere shortening in telomerase-null mice. Am J Physiol Lung Cell Mol Physiol 296(1):L57-70. [PubMed: 18952756]  [MGI Ref ID J:144864]

Lee J; Sung YH; Cheong C; Choi YS; Jeon HK; Sun W; Hahn WC; Ishikawa F; Lee HW. 2008. TERT promotes cellular and organismal survival independently of telomerase activity. Oncogene 27(26):3754-60. [PubMed: 18223679]  [MGI Ref ID J:138281]

Leri A; Franco S; Zacheo A; Barlucchi L; Chimenti S; Limana F; Nadal-Ginard B; Kajstura J; Anversa P; Blasco MA. 2003. Ablation of telomerase and telomere loss leads to cardiac dilatation and heart failure associated with p53 upregulation. EMBO J 22(1):131-9. [PubMed: 12505991]  [MGI Ref ID J:110900]

Liu L; Blasco M; Trimarchi J; Keefe D. 2002. An Essential Role for Functional Telomeres in Mouse Germ Cells during Fertilization and Early Development. Dev Biol 249(1):74. [PubMed: 12217319]  [MGI Ref ID J:78765]

Liu L; Blasco MA; Keefe DL. 2002. Requirement of functional telomeres for metaphase chromosome alignments and integrity of meiotic spindles. EMBO Rep 3(3):230-4. [PubMed: 11882542]  [MGI Ref ID J:108982]

Liu L; DiGirolamo CM; Navarro PA; Blasco MA; Keefe DL. 2004. Telomerase deficiency impairs differentiation of mesenchymal stem cells. Exp Cell Res 294(1):1-8. [PubMed: 14980495]  [MGI Ref ID J:115468]

Liu L; Franco S; Spyropoulos B; Moens PB; Blasco MA; Keefe DL. 2004. Irregular telomeres impair meiotic synapsis and recombination in mice. Proc Natl Acad Sci U S A 101(17):6496-501. [PubMed: 15084742]  [MGI Ref ID J:89751]

Marion RM; Strati K; Li H; Tejera A; Schoeftner S; Ortega S; Serrano M; Blasco MA. 2009. Telomeres acquire embryonic stem cell characteristics in induced pluripotent stem cells. Cell Stem Cell 4(2):141-54. [PubMed: 19200803]  [MGI Ref ID J:149691]

Maser RS; Choudhury B; Campbell PJ; Feng B; Wong KK; Protopopov A; O'Neil J; Gutierrez A; Ivanova E; Perna I; Lin E; Mani V; Jiang S; McNamara K; Zaghlul S; Edkins S; Stevens C; Brennan C; Martin ES; Wiedemeyer R; Kabbarah O; Nogueira C; Histen G; Aster J; Mansour M; Duke V; Foroni L; Fielding AK; Goldstone AH; Rowe JM; Wang YA; Look AT; Stratton MR; Chin L; Futreal PA; DePinho RA. 2007. Chromosomally unstable mouse tumours have genomic alterations similar to diverse human cancers. Nature 447(7147):966-71. [PubMed: 17515920]  [MGI Ref ID J:122751]

Maser RS; Wong KK; Sahin E; Xia H; Naylor M; Hedberg HM; Artandi SE; DePinho RA. 2007. DNA-dependent protein kinase catalytic subunit is not required for dysfunctional telomere fusion and checkpoint response in the telomerase-deficient mouse. Mol Cell Biol 27(6):2253-65. [PubMed: 17145779]  [MGI Ref ID J:118902]

McNees CJ; Tejera AM; Martinez P; Murga M; Mulero F; Fernandez-Capetillo O; Blasco MA. 2010. ATR suppresses telomere fragility and recombination but is dispensable for elongation of short telomeres by telomerase. J Cell Biol 188(5):639-52. [PubMed: 20212315]  [MGI Ref ID J:157978]

Morrish TA; Greider CW. 2009. Short telomeres initiate telomere recombination in primary and tumor cells. PLoS Genet 5(1):e1000357. [PubMed: 19180191]  [MGI Ref ID J:146864]

Munoz P; Blanco R; Flores JM; Blasco MA. 2005. XPF nuclease-dependent telomere loss and increased DNA damage in mice overexpressing TRF2 result in premature aging and cancer. Nat Genet 37(10):1063-71. [PubMed: 16142233]  [MGI Ref ID J:102653]

Nalapareddy K; Choudhury AR; Gompf A; Ju Z; Ravipati S; Leucht T; Lechel A; Rudolph KL. 2010. CHK2-independent induction of telomere dysfunction checkpoints in stem and progenitor cells. EMBO Rep 11(8):619-25. [PubMed: 20577265]  [MGI Ref ID J:168164]

O'Hagan R; Chang S; Maser R; Mohan R; Artandi S; Chin L; DePinho R. 2002. Telomere dysfunction provokes regional amplification and deletion in cancer genomes. Cancer Cell 2(2):149-55. [PubMed: 12204535]  [MGI Ref ID J:78479]

Palacios JA; Herranz D; De Bonis ML; Velasco S; Serrano M; Blasco MA. 2010. SIRT1 contributes to telomere maintenance and augments global homologous recombination. J Cell Biol 191(7):1299-313. [PubMed: 21187328]  [MGI Ref ID J:167988]

Perera SA; Maser RS; Xia H; McNamara K; Protopopov A; Chen L; Hezel AF; Kim CF; Bronson RT; Castrillon DH; Chin L; Bardeesy N; Depinho RA; Wong KK. 2008. Telomere dysfunction promotes genome instability and metastatic potential in a K-ras p53 mouse model of lung cancer. Carcinogenesis 29(4):747-53. [PubMed: 18283039]  [MGI Ref ID J:133322]

Perez-Rivero G; Ruiz-Torres MP; Diez-Marques ML; Canela A; Lopez-Novoa JM; Rodriguez-Puyol M; Blasco MA; Rodriguez-Puyol D. 2008. Telomerase deficiency promotes oxidative stress by reducing catalase activity. Free Radic Biol Med 45(9):1243-51. [PubMed: 18718525]  [MGI Ref ID J:141204]

Perez-Rivero G; Ruiz-Torres MP; Rivas-Elena JV; Jerkic M; Diez-Marques ML; Lopez-Novoa JM; Blasco MA; Rodriguez-Puyol D. 2006. Mice deficient in telomerase activity develop hypertension because of an excess of endothelin production. Circulation 114(4):309-17. [PubMed: 16831983]  [MGI Ref ID J:123085]

Pickett HA; Henson JD; Au AY; Neumann AA; Reddel RR. 2011. Normal mammalian cells negatively regulate telomere length by telomere trimming. Hum Mol Genet 20(23):4684-92. [PubMed: 21903669]  [MGI Ref ID J:177559]

Poch E; Carbonell P; Franco S; Diez-Juan A; Blasco MA; Andres V. 2004. Short telomeres protect from diet-induced atherosclerosis in apolipoprotein E-null mice. FASEB J 18(2):418-20. [PubMed: 14688198]  [MGI Ref ID J:119421]

Qi L; Strong MA; Karim BO; Armanios M; Huso DL; Greider CW. 2003. Short telomeres and ataxia-telangiectasia mutated deficiency cooperatively increase telomere dysfunction and suppress tumorigenesis. Cancer Res 63(23):8188-96. [PubMed: 14678974]  [MGI Ref ID J:87091]

Qi L; Strong MA; Karim BO; Huso DL; Greider CW. 2005. Telomere fusion to chromosome breaks reduces oncogenic translocations and tumour formation. Nat Cell Biol 7(7):706-11. [PubMed: 15965466]  [MGI Ref ID J:100156]

Rai R; Zheng H; He H; Luo Y; Multani A; Carpenter PB; Chang S. 2010. The function of classical and alternative non-homologous end-joining pathways in the fusion of dysfunctional telomeres. EMBO J 29(15):2598-610. [PubMed: 20588252]  [MGI Ref ID J:163387]

Rodriguez S; Goyanes V; Segrelles E; Blasco M; Gosalvez J; Fernandez JL. 2005. Critically short telomeres are associated with sperm DNA fragmentation. Fertil Steril 84(4):843-5. [PubMed: 16213831]  [MGI Ref ID J:106586]

Rudolph KL; Chang S; Lee HW; Blasco M; Gottlieb GJ; Greider C; DePinho RA. 1999. Longevity, stress response, and cancer in aging telomerase-deficient mice. Cell 96(5):701-12. [PubMed: 10089885]  [MGI Ref ID J:53350]

Rudolph KL; Chang S; Millard M; Schreiber-Agus N; DePinho RA. 2000. Inhibition of experimental liver cirrhosis in mice by telomerase gene delivery [see comments] Science 287(5456):1253-8. [PubMed: 10678830]  [MGI Ref ID J:60636]

Rudolph KL; Millard M; Bosenberg MW; DePinho RA. 2001. Telomere dysfunction and evolution of intestinal carcinoma in mice and humans. Nat Genet 28(2):155-9. [PubMed: 11381263]  [MGI Ref ID J:69730]

Sarin KY; Cheung P; Gilison D; Lee E; Tennen RI; Wang E; Artandi MK; Oro AE; Artandi SE. 2005. Conditional telomerase induction causes proliferation of hair follicle stem cells. Nature 436(7053):1048-52. [PubMed: 16107853]  [MGI Ref ID J:100644]

Satyanarayana A; Wiemann SU; Buer J; Lauber J; Dittmar KE; Wustefeld T; Blasco MA; Manns MP; Rudolph KL. 2003. Telomere shortening impairs organ regeneration by inhibiting cell cycle re-entry of a subpopulation of cells. EMBO J 22(15):4003-13. [PubMed: 12881434]  [MGI Ref ID J:84928]

Schaetzlein S; Kodandaramireddy NR; Ju Z; Lechel A; Stepczynska A; Lilli DR; Clark AB; Rudolph C; Kuhnel F; Wei K; Schlegelberger B; Schirmacher P; Kunkel TA; Greenberg RA; Edelmann W; Rudolph KL. 2007. Exonuclease-1 deletion impairs DNA damage signaling and prolongs lifespan of telomere-dysfunctional mice. Cell 130(5):863-77. [PubMed: 17803909]  [MGI Ref ID J:129929]

Schaetzlein S; Lucas-Hahn A; Lemme E; Kues WA; Dorsch M; Manns MP; Niemann H; Rudolph KL. 2004. Telomere length is reset during early mammalian embryogenesis. Proc Natl Acad Sci U S A 101(21):8034-8. [PubMed: 15148368]  [MGI Ref ID J:90665]

Schoeftner S; Blanco R; de Silanes IL; Munoz P; Gomez-Lopez G; Flores JM; Blasco MA. 2009. Telomere shortening relaxes X chromosome inactivation and forces global transcriptome alterations. Proc Natl Acad Sci U S A 106(46):19393-8. [PubMed: 19887628]  [MGI Ref ID J:154764]

Schoeftner S; Blasco MA. 2008. Developmentally regulated transcription of mammalian telomeres by DNA-dependent RNA polymerase II. Nat Cell Biol 10(2):228-36. [PubMed: 18157120]  [MGI Ref ID J:132362]

Sebastian C; Herrero C; Serra M; Lloberas J; Blasco MA; Celada A. 2009. Telomere shortening and oxidative stress in aged macrophages results in impaired STAT5a phosphorylation. J Immunol 183(4):2356-64. [PubMed: 19605693]  [MGI Ref ID J:151571]

Siegl-Cachedenier I; Munoz P; Flores JM; Klatt P; Blasco MA. 2007. Deficient mismatch repair improves organismal fitness and survival of mice with dysfunctional telomeres. Genes Dev 21(17):2234-47. [PubMed: 17785530]  [MGI Ref ID J:125218]

Song Z; Wang J; Guachalla LM; Terszowski G; Rodewald HR; Ju Z; Rudolph KL. 2010. Alterations of the systemic environment are the primary cause of impaired B and T lymphopoiesis in telomere-dysfunctional mice. Blood 115(8):1481-9. [PubMed: 19965646]  [MGI Ref ID J:157779]

Sperka T; Song Z; Morita Y; Nalapareddy K; Guachalla LM; Lechel A; Begus-Nahrmann Y; Burkhalter MD; Mach M; Schlaudraff F; Liss B; Ju Z; Speicher MR; Rudolph KL. 2011. Puma and p21 represent cooperating checkpoints limiting self-renewal and chromosomal instability of somatic stem cells in response to telomere dysfunction. Nat Cell Biol 14(1):73-9. [PubMed: 22138576]  [MGI Ref ID J:178922]

Stout GJ; Blasco MA. 2013. Telomere length and telomerase activity impact the UV sensitivity syndrome xeroderma pigmentosum C. Cancer Res 73(6):1844-54. [PubMed: 23288511]  [MGI Ref ID J:196907]

Tanemura K; Ogura A; Cheong C; Gotoh H; Matsumoto K; Sato E; Hayashi Y; Lee HW; Kondo T. 2005. Dynamic rearrangement of telomeres during spermatogenesis in mice. Dev Biol 281(2):196-207. [PubMed: 15893973]  [MGI Ref ID J:98586]

Thanasoula M; Escandell JM; Martinez P; Badie S; Munoz P; Blasco MA; Tarsounas M. 2010. p53 Prevents entry into mitosis with uncapped telomeres. Curr Biol 20(6):521-6. [PubMed: 20226664]  [MGI Ref ID J:158665]

Tuo B; Ju Z; Riederer B; Engelhardt R; Manns MP; Rudolph KL; Seidler U. 2012. Telomere shortening is associated with reduced duodenal HCOFormula secretory but normal gastric acid secretory capacity in aging mice. Am J Physiol Gastrointest Liver Physiol 303(12):G1312-21. [PubMed: 23019197]  [MGI Ref ID J:193657]

Vidal-Cardenas SL; Greider CW. 2010. Comparing effects of mTR and mTERT deletion on gene expression and DNA damage response: a critical examination of telomere length maintenance-independent roles of telomerase. Nucleic Acids Res 38(1):60-71. [PubMed: 19850716]  [MGI Ref ID J:173011]

Wang J; Sun Q; Morita Y; Jiang H; Gross A; Lechel A; Hildner K; Guachalla LM; Gompf A; Hartmann D; Schambach A; Wuestefeld T; Dauch D; Schrezenmeier H; Hofmann WK; Nakauchi H; Ju Z; Kestler HA; Zender L; Rudolph KL. 2012. A differentiation checkpoint limits hematopoietic stem cell self-renewal in response to DNA damage. Cell 148(5):1001-14. [PubMed: 22385964]  [MGI Ref ID J:190258]

Wang Y; Shen MF; Chang S. 2011. Essential roles for Pot1b in HSC self-renewal and survival. Blood 118(23):6068-77. [PubMed: 21948176]  [MGI Ref ID J:179106]

Westhoff JH; Schildhorn C; Jacobi C; Homme M; Hartner A; Braun H; Kryzer C; Wang C; von Zglinicki T; Kranzlin B; Gretz N; Melk A. 2010. Telomere shortening reduces regenerative capacity after acute kidney injury. J Am Soc Nephrol 21(2):327-36. [PubMed: 19959722]  [MGI Ref ID J:185862]

Wiemann SU; Satyanarayana A; Buer J; Kamino K; Manns MP; Rudolph KL. 2005. Contrasting effects of telomere shortening on organ homeostasis, tumor suppression, and survival during chronic liver damage. Oncogene 24(9):1501-9. [PubMed: 15608677]  [MGI Ref ID J:96885]

Wong KK; Chang S; Weiler SR; Ganesan S; Chaudhuri J; Zhu C; Artandi SE; Rudolph KL; Gottlieb GJ; Chin L; Alt FW; DePinho RA. 2000. Telomere dysfunction impairs DNA repair and enhances sensitivity to ionizing radiation Nat Genet 26(1):85-8. [PubMed: 10973255]  [MGI Ref ID J:64366]

Wong KK; Maser RS; Bachoo RM; Menon J; Carrasco DR; Gu Y; Alt FW; DePinho RA. 2003. Telomere dysfunction and Atm deficiency compromises organ homeostasis and accelerates ageing. Nature 421(6923):643-8. [PubMed: 12540856]  [MGI Ref ID J:81552]

von Figura G; Wagner M; Nalapareddy K; Hartmann D; Kleger A; Guachalla LM; Rolyan H; Adler G; Rudolph KL. 2011. Regeneration of the exocrine pancreas is delayed in telomere-dysfunctional mice. PLoS One 6(2):e17122. [PubMed: 21364961]  [MGI Ref ID J:171067]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX11

Colony Maintenance

Breeding & HusbandryThe Dmdmdx-4Cv mutation is X-linked.

Because telomere length is progressively shortened with successive generations of breeding homozygous mTR null mice (mTR-/-) together, several generations of animals may be available from The Jackson Laboratory Repository as defined below:

A) G0 animals are dystrophin-deficient and heterozygous for mTR. G0 animals have the same phenotype as C57BL/6J-congenic Dmdmdx-4Cv homozygous mice (Stock No. 002378). Breeding G0 animals together (Dmdmdx-4Cv/mdx-4Cv;mTR+/- females and Dmdmdx-4Cv/Y;mTR+/- males) results in the following offspring:
i) Dmdmdx-4Cv/mdx-4Cv;mTR+/+ females
ii) Dmdmdx-4Cv/Y;mTR+/+ males
iii) Dmdmdx-4Cv/mdx-4Cv;mTR+/- females (G0 females)
iv) Dmdmdx-4Cv/Y;mTR+/- males (G0 males)
v) Dmdmdx-4Cv/mdx-4Cv;mTR-/- females (G1 females)
vi) Dmdmdx-4Cv/Y;mTR-/- males (G1 males)

B) G1 animals are the first generation of mice deficient in both dystrophin and mTR. G1 animals have enhanced DMD myopathology. Breeding G1 animals together (Dmdmdx-4Cv/mdx-4Cv;mTR-/- females and Dmdmdx-4Cv/Y;mTR-/- males) results in the following offspring:
i) Dmdmdx-4Cv/mdx-4Cv;mTR-/- females (G2 females)
ii) Dmdmdx-4Cv/Y;mTR-/- males (G2 males)

C) G2 animals are the second generation of mice deficient in both mdx and mTR. G2 animals have enhanced DMD myopathology that is more severe than G1 animals. Breeding G2 animals together results in G3 offspring.

For routine maintenance of the living mdx/mTR colony at The Jackson Laboratory Repository, we breed G0 mice together. By this method, the animals in part A (i-vi) may be available. Please inquire for the availability of G2 animals (if any).

Mating SystemSee Colony Maintenance under the Health & Care tab         (Female x Male)   04-APR-14

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 $232.00MaleHeterozygous for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  
$232.00FemaleHeterozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Individual Mouse $232.00MaleHomozygous for Terctm1Rdp , Hemizygous for Dmdmdx-4Cv  
$232.00FemaleHomozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Individual Mouse $232.00MaleWild-type for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  
$232.00FemaleWild-type for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Price per Pair (US dollars $)Pair Genotype
$464.00Heterozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv x Heterozygous for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  

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 $301.60MaleHeterozygous for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  
$301.60FemaleHeterozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Individual Mouse $301.60MaleHomozygous for Terctm1Rdp , Hemizygous for Dmdmdx-4Cv  
$301.60FemaleHomozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Individual Mouse $301.60MaleWild-type for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  
$301.60FemaleWild-type for Terctm1Rdp, Homozygous for Dmdmdx-4Cv  
Price per Pair (US dollars $)Pair Genotype
$603.20Heterozygous for Terctm1Rdp, Homozygous for Dmdmdx-4Cv x Heterozygous for Terctm1Rdp, Hemizygous for Dmdmdx-4Cv  

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
   See control note: Depending upon the nature of the experiment, the following mouse line(s) may be appropriate controls:
C57BL/6J.mTR-/- (Stock No. 004132: B6.Cg-Terctm1Rdp/J)
and/or B6.mdx-4Cv (Stock No. 002378: B6Ros.Cg-Dmdmdx-4Cv/J)
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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

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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.

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