Strain Name:

B6.129S1(Cg)-Lmnatm1Stw/BkknJ

Stock Number:

009125

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Availability:

Repository- Live

Use Restrictions Apply, see Terms of Use
Mice harboring the lamin A/C (Lmna) null mutation are a model for the autosomal variant of Emery-Dreifuss muscular dystrophy (EDMD) and may be useful in studying the role of lamins, inner nuclear membrane proteins, nuclear envelope integrity, and chromatin domain anchoring sites in EDMD.

Description

Strain Information

Type Congenic; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating System+/+ sibling x Heterozygote         (Female x Male)   29-DEC-09
Specieslaboratory mouse
GenerationN10+F9 (11-DEC-13)
Generation Definitions
 
Donating Investigator Brian K Kennedy,   University of Washington

Description
Mice heterozygotes for this lamin A/C mutation are viable and fertile. The targeted allele does not express both full-length transcripts or stable lamin A/C protein. Homozygotes (Lmna -/- mice) exhibit severely retarded postnatal growth beginning as early as 2 weeks of age and abnormal movement/gait by 3-4 weeks of age that progresses to distinct scoliosis/kyphosis and death around 8 weeks of age. Lmna -/- mice also have tissue-specific alterations of nuclear envelope integrity and mislocalization of the inner nuclear membrane protein emerin. In skeletal and cardiac muscle, this results in rapid myopathic onset closely resembling Emery-Dreifuss muscular dystrophy (EDMD). These mice are a model for the autosomal variant of EDMD and may be useful in studying the role of lamins, inner nuclear membrane proteins, nuclear envelope integrity, and chromatin domain anchoring sites in EDMD.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

Development
These lamin A/C-mutant mice were published in 1999 and generated by the laboratory of Dr. Colin L. Stewart (National Cancer Institute-Frederick Cancer Research and Development Center, Maryland). A targeting vector was designed to replace exon 8 through part of exon 11 of the lamin A/C (Lmna) locus with a reverse-oriented PGK-neomycin resistance cassette. This removed 114 codons as well as the 3' UTR, including the polyadenylation signal of lamin C, whereas 152 codons were eliminated from the lamin A coding region. The targeting construct was electroporated into 129S1/Sv-Oca2+ Tyr+ Kitl+-derived W9.5 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts and the resulting mutant mice were maintained on an undisclosed genetic background. In 2006, Lmna-mutant mice were sent to the laboratory of Brian K. Kennedy (University of Washington, Seattle) where they were subsequently backcrossed to C57BL/6J inbred mice for at least nine generations prior to sending to The Jackson Laboratory. Upon arrival, mice were bred with C57BL/6J inbred mice (Stock No. 000664) for at least one generation to establish the mutant colony. The Y chromosome may not have been fixed to the genetic background during the backcross.

Control Information

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

Related Strains

Strains carrying other alleles of Lmna
001934   B6(D2)-LmnaDhe/TyGrsrJ
View Strains carrying other alleles of Lmna     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Cardiomyopathy, Dilated, 1A; CMD1A
Charcot-Marie-Tooth Disease, Axonal, Type 2B1; CMT2B1
Emery-Dreifuss Muscular Dystrophy 2, Autosomal Dominant; EDMD2
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Lipodystrophy, Familial Partial, Type 2; FPLD2
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Cardiomyopathy, Dilated, with Hypergonadotropic Hypogonadism   (LMNA)
Heart-Hand Syndrome, Slovenian Type   (LMNA)
Hutchinson-Gilford Progeria Syndrome; HGPS   (LMNA)
Mandibuloacral Dysplasia with Type A Lipodystrophy; MADA   (LMNA)
Muscular Dystrophy, Congenital, Lmna-Related   (LMNA)
Muscular Dystrophy, Limb-Girdle, Type 1B; LGMD1B   (LMNA)
Restrictive Dermopathy, Lethal   (LMNA)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Lmnatm1Stw/Lmna+

        involves: 129S1/Sv
  • cellular phenotype
  • abnormal cell nucleus morphology
    • in many MEFs, nuclei appear elongated but distribution of nuclear envelope proteins is normal except for increased levels of cytoplasmic emerin   (MGI Ref ID J:58702)

Lmnatm1Stw/Lmna+

        involves: 129S1/Sv * C57BL/6J
  • homeostasis/metabolism phenotype
  • increased circulating insulin level
    • on a high fat diet, females develop mild insulin resistance that correlates with increased body weight, no insulin resistance is seen in males   (MGI Ref ID J:75101)
    • on a normal diet, serum glucose, insulin, and triglyceride levels are normal, insulin sensitivity and glucose tolerance   (MGI Ref ID J:75101)
  • insulin resistance
    • on a high fat diet, females develop increased insulin but not glucose levels   (MGI Ref ID J:75101)
  • adipose tissue phenotype
  • *normal* adipose tissue phenotype
    • no signs of lipoatrophy are seen   (MGI Ref ID J:75101)
  • liver/biliary system phenotype
  • *normal* liver/biliary system phenotype
    • liver morphology is normal   (MGI Ref ID J:75101)

Lmnatm1Stw/Lmnatm1Stw

        involves: 129S1/Sv
  • mortality/aging
  • premature death
    • all die by 8 weeks of age   (MGI Ref ID J:58702)
  • cellular phenotype
  • abnormal cell nucleus morphology
    • in MEFs, nuclei appear elongated or irregular with loss of B-type lamins from 1 pole, slight clustering of nuclear pore complexes, and loss of emerin from the nuclear envelope into the cytoplasm   (MGI Ref ID J:58702)
    • in MEFs and hepatocytes, patchy thinning or loss of heterochromatin from the nuclear face of the inner nuclear membrane is seen and these regions also lack identifiable nuclear pore complexes   (MGI Ref ID J:58702)
    • the nuclear envelope is more easily fragmented during isolation   (MGI Ref ID J:58702)
  • growth/size/body phenotype
  • decreased body weight
    • at 4 weeks, mean body weight is about 50% of wild-type and heterozygous littermates   (MGI Ref ID J:58702)
  • postnatal growth retardation
    • growth retardation is seen by 2-3 weeks of age and growth ceases by 4 weeks of age   (MGI Ref ID J:58702)
  • muscle phenotype
  • dystrophic muscle
    • muscles surrounding the femur and perivertebral muscles are dystrophic while cardiac muscle is variably affected with ventricular muscle more severely impaired; however, muscles in the head, tongue and diaphragm are relatively normal   (MGI Ref ID J:58702)
    • muscle fibers are variably affected with those closer to the bone being more severely impaired   (MGI Ref ID J:58702)
    • no elevation of serum creatine kinase levels are detected   (MGI Ref ID J:58702)
  • muscle degeneration
    • in the heart, some ventricular myocytes show signs of degeneration often associated with patchy mineralization   (MGI Ref ID J:58702)
  • behavior/neurological phenotype
  • abnormal gait
    • at 3-4 weeks mice display a stiff walking posture with splayed hind legs   (MGI Ref ID J:58702)
  • decreased grip strength
    • at 3-4 weeks mice have reduced grip strength   (MGI Ref ID J:58702)
  • skeleton phenotype
  • kyphoscoliosis
    • progressive kyphoscoliosis starting around 3-4 weeks of age   (MGI Ref ID J:58702)
  • adipose tissue phenotype
  • decreased white adipose tissue amount
    • absence of white fat   (MGI Ref ID J:58702)
  • nervous system phenotype
  • abnormal axon morphology
    • sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are present   (MGI Ref ID J:75378)
  • abnormal myelination
    • nonmyelinated axons are present in the sciatic nerve   (MGI Ref ID J:75378)
  • abnormal sciatic nerve morphology
    • sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are presen   (MGI Ref ID J:75378)
  • immune system phenotype
  • small spleen
    • probably secondary to physiological stress   (MGI Ref ID J:58702)
  • thymus atrophy
    • probably secondary to physiological stress   (MGI Ref ID J:58702)
  • hematopoietic system phenotype
  • small spleen
    • probably secondary to physiological stress   (MGI Ref ID J:58702)
  • thymus atrophy
    • probably secondary to physiological stress   (MGI Ref ID J:58702)

Lmnatm1Stw/Lmnatm1Stw

        involves: 129S1/Sv * C57BL/6
  • mortality/aging
  • premature death
    • die by 4-6 weeks of age   (MGI Ref ID J:95274)
  • growth/size/body phenotype
  • postnatal growth retardation   (MGI Ref ID J:95274)
  • muscle phenotype
  • muscle weakness   (MGI Ref ID J:95274)
  • reproductive system phenotype
  • arrest of male meiosis
    • increased frequency of leptotene and zygotene spermatocytes and decreased frequency of pachytene and diplotene spermatocytes resulting from arrest and apoptosis of spermatocytes during the pachytene stage   (MGI Ref ID J:88595)
    • however, oogenesis in females is normal   (MGI Ref ID J:88595)
    • unpaired chromosomes and/or improperly paired sex chromosomes and failure to progress through prophase I are seen   (MGI Ref ID J:88595)
  • azoospermia
    • spermatids and spermatozoa are largely absent   (MGI Ref ID J:88595)
  • small seminiferous tubules   (MGI Ref ID J:88595)
  • small testis   (MGI Ref ID J:88595)
  • endocrine/exocrine gland phenotype
  • small seminiferous tubules   (MGI Ref ID J:88595)
  • small testis   (MGI Ref ID J:88595)

Lmnatm1Stw/Lmnatm1Stw

        involves: 129S1/Sv * C57BL/6J
  • homeostasis/metabolism phenotype
  • decreased circulating glucose level
    • at 4 weeks serum glucose levels are decreased however insulin levels are only slightly and not significantly decreased   (MGI Ref ID J:75101)
  • decreased circulating triglyceride level
    • only significant in 4 week old females although levels in males also tend to be lower   (MGI Ref ID J:75101)
View Research Applications

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

Cardiovascular Research
Heart Abnormalities

Cell Biology Research
Cell Cycle Regulation
Genes Regulating Growth and Proliferation

Developmental Biology Research
Growth Defects
Internal/Organ Defects
      heart
Perinatal Lethality
      Homozygous

Internal/Organ Research
Heart Abnormalities
Other

Neurobiology Research
Muscular Dystrophy
      Emery-Dreifuss type

Research Tools
Cardiovascular Research
Cell Biology Research
Developmental Biology Research
Internal/Organ Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Lmnatm1Stw
Allele Name targeted mutation 1, Colin L Stewart
Allele Type Targeted (knock-out)
Common Name(s) Lmna-;
Mutation Made By Colin Stewart,   Institute of Medical Biology
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 Lmna, lamin A
Chromosome 3
Gene Common Name(s) CDCD1; CDDC; CMD1A; CMT2B1; Dhe; EMD2; FPL; FPLD; FPLD2; HGPS; IDC; LDP1; LFP; LGMD1B; LMN1; LMNC; LMNL1; PRO1; disheveled hair and ear; lamin A/C;
Molecular Note A PGK-neomycin resistance cassette replaced a genomic segment containing exons 8-10. Western blot analysis on extracts of both embryonic fibroblasts and liver nuclear membranes from homozygous mice confirmed that no detectable protein was expressed fromthis allele. [MGI Ref ID J:58702]

Genotyping

Genotyping Information

Genotyping Protocols

Lmnatm1Stw,

MELT


Lmnatm1Stw, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Frock RL; Kudlow BA; Evans AM; Jameson SA; Hauschka SD; Kennedy BK. 2006. Lamin A/C and emerin are critical for skeletal muscle satellite cell differentiation. Genes Dev 20(4):486-500. [PubMed: 16481476]  [MGI Ref ID J:105720]

Sullivan T; Escalante-Alcalde D; Bhatt H; Anver M; Bhat N; Nagashima K; Stewart CL; Burke B. 1999. Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy. J Cell Biol 147(5):913-20. [PubMed: 10579712]  [MGI Ref ID J:58702]

Additional References

Lmnatm1Stw related

Alsheimer M; Liebe B; Sewell L; Stewart CL; Scherthan H; Benavente R. 2004. Disruption of spermatogenesis in mice lacking A-type lamins. J Cell Sci 117(Pt 7):1173-8. [PubMed: 14996939]  [MGI Ref ID J:88595]

Broers JL; Peeters EA; Kuijpers HJ; Endert J; Bouten CV; Oomens CW; Baaijens FP; Ramaekers FC. 2004. Decreased mechanical stiffness in LMNA-/- cells is caused by defective nucleo-cytoskeletal integrity: implications for the development of laminopathies. Hum Mol Genet 13(21):2567-80. [PubMed: 15367494]  [MGI Ref ID J:94403]

Chandar S; Yeo LS; Leimena C; Tan JC; Xiao XH; Nikolova-Krstevski V; Yasuoka Y; Gardiner-Garden M; Wu J; Kesteven S; Karlsdotter L; Natarajan S; Carlton A; Rainer S; Feneley MP; Fatkin D. 2010. Effects of mechanical stress and carvedilol in lamin A/C-deficient dilated cardiomyopathy. Circ Res 106(3):573-82. [PubMed: 20019332]  [MGI Ref ID J:170882]

Chen CY; Chi YH; Mutalif RA; Starost MF; Myers TG; Anderson SA; Stewart CL; Jeang KT. 2012. Accumulation of the inner nuclear envelope protein Sun1 is pathogenic in progeric and dystrophic laminopathies. Cell 149(3):565-77. [PubMed: 22541428]  [MGI Ref ID J:186197]

Coffinier C; Jung HJ; Li Z; Nobumori C; Yun UJ; Farber EA; Davies BS; Weinstein MM; Yang SH; Lammerding J; Farahani JN; Bentolila LA; Fong LG; Young SG. 2010. Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice. J Biol Chem 285(27):20818-26. [PubMed: 20439468]  [MGI Ref ID J:165928]

Cohen TV; Gnocchi VF; Cohen JE; Phadke A; Liu H; Ellis JA; Foisner R; Stewart CL; Zammit PS; Partridge TA. 2013. Defective skeletal muscle growth in lamin A/C-deficient mice is rescued by loss of Lap2alpha. Hum Mol Genet 22(14):2852-69. [PubMed: 23535822]  [MGI Ref ID J:198543]

Cutler DA; Sullivan T; Marcus-Samuels B; Stewart CL; Reitman ML. 2002. Characterization of adiposity and metabolism in Lmna-deficient mice. Biochem Biophys Res Commun 291(3):522-7. [PubMed: 11855819]  [MGI Ref ID J:75101]

De Sandre-Giovannoli A; Chaouch M; Kozlov S; Vallat JM; Tazir M; Kassouri N; Szepetowski P; Hammadouche T; Vandenberghe A; Stewart CL; Grid D; Levy N. 2002. Homozygous defects in LMNA, encoding lamin A/C nuclear-envelope proteins, cause autosomal recessive axonal neuropathy in human (Charcot-Marie-Tooth disorder type 2) and mouse. Am J Hum Genet 70(3):726-36. [PubMed: 11799477]  [MGI Ref ID J:75378]

Folker ES; Ostlund C; Luxton GW; Worman HJ; Gundersen GG. 2011. Lamin A variants that cause striated muscle disease are defective in anchoring transmembrane actin-associated nuclear lines for nuclear movement. Proc Natl Acad Sci U S A 108(1):131-6. [PubMed: 21173262]  [MGI Ref ID J:169002]

Fong LG; Ng JK; Lammerding J; Vickers TA; Meta M; Cote N; Gavino B; Qiao X; Chang SY; Young SR; Yang SH; Stewart CL; Lee RT; Bennett CF; Bergo MO; Young SG. 2006. Prelamin A and lamin A appear to be dispensable in the nuclear lamina. J Clin Invest 116(3):743-52. [PubMed: 16511604]  [MGI Ref ID J:106473]

Fong LG; Ng JK; Meta M; Cote N; Yang SH; Stewart CL; Sullivan T; Burghardt A; Majumdar S; Reue K; Bergo MO; Young SG. 2004. Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice. Proc Natl Acad Sci U S A 101(52):18111-6. [PubMed: 15608054]  [MGI Ref ID J:95274]

Frock RL; Chen SC; Da DF; Frett E; Lau C; Brown C; Pak DN; Wang Y; Muchir A; Worman HJ; Santana LF; Ladiges WC; Rabinovitch PS; Kennedy BK. 2012. Cardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- mice. PLoS One 7(8):e42918. [PubMed: 22905185]  [MGI Ref ID J:189904]

Gnocchi VF; Scharner J; Huang Z; Brady K; Lee JS; White RB; Morgan JE; Sun YB; Ellis JA; Zammit PS. 2011. Uncoordinated transcription and compromised muscle function in the lmna-null mouse model of emery-dreifuss muscular dystrophy. PLoS One 6(2):e16651. [PubMed: 21364987]  [MGI Ref ID J:171065]

Gonzalez-Suarez I; Redwood AB; Grotsky DA; Neumann MA; Cheng EH; Stewart CL; Dusso A; Gonzalo S. 2011. A new pathway that regulates 53BP1 stability implicates Cathepsin L and vitamin D in DNA repair. EMBO J 30(16):3383-96. [PubMed: 21750527]  [MGI Ref ID J:176746]

Hale JS; Frock RL; Mamman SA; Fink PJ; Kennedy BK. 2010. Cell-extrinsic defective lymphocyte development in Lmna(-/-) mice. PLoS One 5(4):e10127. [PubMed: 20405040]  [MGI Ref ID J:160149]

Haque F; Lloyd DJ; Smallwood DT; Dent CL; Shanahan CM; Fry AM; Trembath RC; Shackleton S. 2006. SUN1 interacts with nuclear lamin A and cytoplasmic nesprins to provide a physical connection between the nuclear lamina and the cytoskeleton. Mol Cell Biol 26(10):3738-51. [PubMed: 16648470]  [MGI Ref ID J:109072]

Ho CY; Jaalouk DE; Vartiainen MK; Lammerding J. 2013. Lamin A/C and emerin regulate MKL1-SRF activity by modulating actin dynamics. Nature 497(7450):507-11. [PubMed: 23644458]  [MGI Ref ID J:198746]

Ji JY; Lee RT; Vergnes L; Fong LG; Stewart CL; Reue K; Young SG; Zhang Q; Shanahan CM; Lammerding J. 2007. Cell nuclei spin in the absence of lamin b1. J Biol Chem 282(27):20015-26. [PubMed: 17488709]  [MGI Ref ID J:123495]

Khatau SB; Hale CM; Stewart-Hutchinson PJ; Patel MS; Stewart CL; Searson PC; Hodzic D; Wirtz D. 2009. A perinuclear actin cap regulates nuclear shape. Proc Natl Acad Sci U S A 106(45):19017-22. [PubMed: 19850871]  [MGI Ref ID J:154670]

Li W; Yeo LS; Vidal C; McCorquodale T; Herrmann M; Fatkin D; Duque G. 2011. Decreased bone formation and osteopenia in lamin a/c-deficient mice. PLoS One 6(4):e19313. [PubMed: 21547077]  [MGI Ref ID J:172372]

Marino G; Ugalde AP; Salvador-Montoliu N; Varela I; Quiros PM; Cadinanos J; van der Pluijm I; Freije JM; Lopez-Otin C. 2008. Premature aging in mice activates a systemic metabolic response involving autophagy induction. Hum Mol Genet 17(14):2196-211. [PubMed: 18443001]  [MGI Ref ID J:136907]

Mejat A; Decostre V; Li J; Renou L; Kesari A; Hantai D; Stewart CL; Xiao X; Hoffman E; Bonne G; Misteli T. 2009. Lamin A/C-mediated neuromuscular junction defects in Emery-Dreifuss muscular dystrophy. J Cell Biol 184(1):31-44. [PubMed: 19124654]  [MGI Ref ID J:146197]

Melcon G; Kozlov S; Cutler DA; Sullivan T; Hernandez L; Zhao P; Mitchell S; Nader G; Bakay M; Rottman JN; Hoffman EP; Stewart CL. 2006. Loss of emerin at the nuclear envelope disrupts the Rb1/E2F and MyoD pathways during muscle regeneration. Hum Mol Genet 15(4):637-51. [PubMed: 16403804]  [MGI Ref ID J:106760]

Nikolova V; Leimena C; McMahon AC; Tan JC; Chandar S; Jogia D; Kesteven SH; Michalicek J; Otway R; Verheyen F; Rainer S; Stewart CL; Martin D; Feneley MP; Fatkin D. 2004. Defects in nuclear structure and function promote dilated cardiomyopathy in lamin A/C-deficient mice. J Clin Invest 113(3):357-69. [PubMed: 14755333]  [MGI Ref ID J:87613]

Nikolova-Krstevski V; Leimena C; Xiao XH; Kesteven S; Tan JC; Yeo LS; Yu ZY; Zhang Q; Carlton A; Head S; Shanahan C; Feneley MP; Fatkin D. 2011. Nesprin-1 and actin contribute to nuclear and cytoskeletal defects in lamin A/C-deficient cardiomyopathy. J Mol Cell Cardiol 50(3):479-86. [PubMed: 21156181]  [MGI Ref ID J:171019]

Ramos FJ; Chen SC; Garelick MG; Dai DF; Liao CY; Schreiber KH; Mackay VL; An EH; Strong R; Ladiges WC; Rabinovitch PS; Kaeberlein M; Kennedy BK. 2012. Rapamycin Reverses Elevated mTORC1 Signaling in Lamin A/C-Deficient Mice, Rescues Cardiac and Skeletal Muscle Function, and Extends Survival. Sci Transl Med 4(144):144ra103. [PubMed: 22837538]  [MGI Ref ID J:186773]

Razafsky D; Blecher N; Markov A; Stewart-Hutchinson PJ; Hodzic D. 2012. LINC complexes mediate the positioning of cone photoreceptor nuclei in mouse retina. PLoS One 7(10):e47180. [PubMed: 23071752]  [MGI Ref ID J:192230]

Varela I; Cadinanos J; Pendas AM; Gutierrez-Fernandez A; Folgueras AR; Sanchez LM; Zhou Z; Rodriguez FJ; Stewart CL; Vega JA; Tryggvason K; Freije JM; Lopez-Otin C. 2005. Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation. Nature 437(7058):564-8. [PubMed: 16079796]  [MGI Ref ID J:134546]

Wang Y; Panteleyev AA; Owens DM; Djabali K; Stewart CL; Worman HJ. 2008. Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin. Hum Mol Genet 17(15):2357-69. [PubMed: 18442998]  [MGI Ref ID J:137663]

Wolf CM; Wang L; Alcalai R; Pizard A; Burgon PG; Ahmad F; Sherwood M; Branco DM; Wakimoto H; Fishman GI; See V; Stewart CL; Conner DA; Berul CI; Seidman CE; Seidman JG. 2008. Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease. J Mol Cell Cardiol 44(2):293-303. [PubMed: 18182166]  [MGI Ref ID J:131905]

Yang SH; Meta M; Qiao X; Frost D; Bauch J; Coffinier C; Majumdar S; Bergo MO; Young SG; Fong LG. 2006. A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation. J Clin Invest 116(8):2115-21. [PubMed: 16862216]  [MGI Ref ID J:113119]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX10

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, heterozygous mice may be bred to wildtype siblings or C57BL/6J inbred mice (Stock No. 000664). Homozygous mice rapidly develop muscular dystrophy; manifest around 3-4 weeks of age with abnormal movement and gait, which progresses to distinct scoliosis/kyphosis and death around 8 weeks of age.
Mating System+/+ sibling x Heterozygote         (Female x Male)   29-DEC-09
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $232.00Female or MaleHeterozygous for Lmnatm1Stw  
Price per Pair (US dollars $)Pair Genotype
$302.00Heterozygous for Lmnatm1Stw x Wild-type for Lmnatm1Stw  
$302.00Wild-type for Lmnatm1Stw x Heterozygous for Lmnatm1Stw  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $301.60Female or MaleHeterozygous for Lmnatm1Stw  
Price per Pair (US dollars $)Pair Genotype
$392.60Heterozygous for Lmnatm1Stw x Wild-type for Lmnatm1Stw  
$392.60Wild-type for Lmnatm1Stw x Heterozygous for Lmnatm1Stw  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Control Information

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

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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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Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

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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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