Strain Name:

FVB-Tg(MECP2)1Hzo/J

Stock Number:

008679

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Availability:

Cryopreserved - Ready for recovery

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As hemizygotes, these human methyl CpG binding protein 2 transgenic mice express the gene at ~2-fold wildtype levels in the brain. Enhanced motor and contextual learning, and enhanced hippocampal synaptic plasticity phenotypes begin around 10 weeks of age. After 20 weeks of age, the mice progressively develop neurological impairments, seizures, become hypoactive (characterized by a freezing-like behavior), and ~30% die by one year of age. Forepaw clasping, aggressiveness, and kyphosis are also characteristic in these mice. This strain may be useful in studies of X-linked delayed-onset neurobehavioral disorders, including MECP2 duplication syndrome, mental retardation, and autism.

Description

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

Strain Information

Type Coisogenic; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating Investigator Huda Zoghbi,   Baylor College of Medicine

Description
As hemizygotes, these human methyl CpG binding protein 2 transgenic mice express the gene at ~2-fold wildtype levels in the brain. Enhanced motor and contextual learning, and enhanced hippocampal synaptic plasticity phenotypes begin around 10 weeks of age. After 20 weeks of age, the mice progressively develop neurological impairments, seizures, become hypoactive (characterized by a freezing-like behavior), and ~30% die by one year of age. Forepaw clasping, aggressiveness, and kyphosis are also characteristic in these mice. This strain may be useful in studies of X-linked delayed-onset neurobehavioral disorders, including MECP2 duplication syndrome, mental retardation, and autism.

Development
A full-length copy of the human gene was injected into FVB fertilized oocytes. The TG1 line was maintained on the FVB background by the donating investigator.

Control Information

  Control
   Noncarrier
   001800 FVB/NJ
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of MECP2
008680   FVB-Tg(MECP2)3Hzo/J
View Strains carrying other alleles of MECP2     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Lubs X-Linked Mental Retardation Syndrome; MRXSL
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Angelman Syndrome; AS   (MECP2)
Autism, Susceptibility to, X-Linked 3; AUTSX3   (MECP2)
Encephalopathy, Neonatal Severe, Due to Mecp2 Mutations   (MECP2)
Mental Retardation, X-Linked, Syndromic 13; MRXS13   (MECP2)
Rett Syndrome; RTT   (MECP2)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(MECP2)1Hzo/0

        involves: FVB
  • mortality/aging
  • premature death
    • 30% of mice die between 20 weeks and 1 year of age; mice surviving past 1 year of age have a normal lifespan   (MGI Ref ID J:94407)
  • nervous system phenotype
  • *normal* nervous system phenotype
    • no alteration in synaptic release probability is detected in neurons   (MGI Ref ID J:126964)
    • abnormal excitatory postsynaptic currents
      • evoked EPSC amplitudes show a 116% enhancement compared to wild-type   (MGI Ref ID J:126964)
    • abnormal miniature excitatory postsynaptic currents
      • mEPSCs show a significant increase in frequency compared to wild-type   (MGI Ref ID J:126964)
    • abnormal synaptic vesicle number
      • neurons exhibit a 104% enhancement in RRP charge relative to wild-type   (MGI Ref ID J:126964)
    • clonic seizures
      • visually observed as early as 20 weeks of age, with increased frequency by 50 weeks   (MGI Ref ID J:94407)
    • enhanced long term potentiation
      • robust enhancement is observed in mutants   (MGI Ref ID J:94407)
    • enhanced paired-pulse facilitation
      • PPF is greatly enhanced in transgenic mice   (MGI Ref ID J:94407)
    • increased CNS synapse formation
      • in vitro, density of synaptic markers is increased by 60%   (MGI Ref ID J:126964)
      • in vivo, at 2 weeks postnatal, number of glutamatergic synapses is increased by 80%; by 5 weeks, difference is much smaller but is still slightly significant   (MGI Ref ID J:126964)
  • behavior/neurological phenotype
  • abnormal contextual conditioning behavior
    • in conditioned fear analysis, mutants and wild-type show similar results in cued fear conditioning, but in contextual conditioning, mutants show elevated freezing behavior indicating greater hippocampal learning   (MGI Ref ID J:94407)
  • abnormal motor coordination/ balance
    • in dowel test, most animals remain inactive on wooden rod the entire trial period   (MGI Ref ID J:94407)
    • enhanced coordination
      • at 10 weeks of age, transgenics perform better on a rotating rod test on day 2 of training relative to wild-type; upon retesting at 20 weeks, mutants perform significantly better than wild-type   (MGI Ref ID J:94407)
  • clonic seizures
    • visually observed as early as 20 weeks of age, with increased frequency by 50 weeks   (MGI Ref ID J:94407)
  • decreased anxiety-related response
    • mice show lower anxiety evidenced by increased vertical activity in open field test   (MGI Ref ID J:94407)
  • hypoactivity
    • in dowel test, transgenic animals display significantly reduced movement and walk off the length of an elevated dowel fewer times than wild-type   (MGI Ref ID J:94407)
    • characterized by freezing behavior   (MGI Ref ID J:94407)
  • increased aggression
    • increased propensity to bite   (MGI Ref ID J:94407)
  • increased vertical activity
    • mice show increased vertical activity compared to controls at 10 and 20 weeks   (MGI Ref ID J:94407)
  • limb grasping
    • forepaw clasping when lifted by tail   (MGI Ref ID J:94407)
  • skeleton phenotype
  • kyphosis   (MGI Ref ID J:94407)

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

Tg(MECP2)1Hzo/0

        either: (FVB/N x 129S6/SvEvTac)F1 or (FVB/N x C57BL/6J)F1
  • behavior/neurological phenotype
  • abnormal social investigation
    • mutants are less interested in their familiar or novel partner mice in the partition test for social interaction and recognition   (MGI Ref ID J:181213)
    • in the three-chamber test for sociability, mutants show a deficit in social approach behavior toward novel partner mice, without a deficit in interest toward a novel object or a deficit in activity or preference for either chamber   (MGI Ref ID J:181213)
  • increased anxiety-related response
    • mutants exhibit anxiety-like behavior in the elevated plus maze   (MGI Ref ID J:181213)
    • mutants spend less time in the lit compartment of the light/dark box compared to wild-type mice indicating anxiety-like behavior   (MGI Ref ID J:181213)
  • homeostasis/metabolism phenotype
  • abnormal corticosterone level
    • stress-induced corticosterone levels are higher in mutants than wild-type mice   (MGI Ref ID J:181213)
View Research Applications

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

Neurobiology Research
Behavioral and Learning Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(MECP2)1Hzo
Allele Name transgene insertion 1, Huda Y Zoghbi
Allele Type Transgenic (Humanized sequence, Inserted expressed sequence)
Common Name(s) MeCP2Tg1; Tg1;
Mutation Made By Huda Zoghbi,   Baylor College of Medicine
Strain of OriginFVB
Expressed Gene MECP2, methyl CpG binding protein 2, human
Promoter MECP2, methyl CpG binding protein 2, human
Molecular Note A full-length copy of the human gene was injected into FVB fertilized oocytes. Line 1 (2-fold higher levels of protein compared to endogenous Mecp2) was established. [MGI Ref ID J:94407]
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(MECP2),

MELT


Tg(MECP2),

SEPARATED MELT


Tg(MECP2), Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Tg(MECP2)1Hzo related

Chahrour M; Jung SY; Shaw C; Zhou X; Wong ST; Qin J; Zoghbi HY. 2008. MeCP2, a key contributor to neurological disease, activates and represses transcription. Science 320(5880):1224-9. [PubMed: 18511691]  [MGI Ref ID J:136439]

Chao HT; Zoghbi HY; Rosenmund C. 2007. MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron 56(1):58-65. [PubMed: 17920015]  [MGI Ref ID J:126964]

Collins AL; Levenson JM; Vilaythong AP; Richman R; Armstrong DL; Noebels JL; David Sweatt J; Zoghbi HY. 2004. Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet 13(21):2679-89. [PubMed: 15351775]  [MGI Ref ID J:94407]

Jiang M; Ash RT; Baker SA; Suter B; Ferguson A; Park J; Rudy J; Torsky SP; Chao HT; Zoghbi HY; Smirnakis SM. 2013. Dendritic arborization and spine dynamics are abnormal in the mouse model of MECP2 duplication syndrome. J Neurosci 33(50):19518-33. [PubMed: 24336718]  [MGI Ref ID J:204660]

Kim S; Chahrour M; Ben-Shachar S; Lim J. 2013. Ube3a/E6AP is involved in a subset of MeCP2 functions. Biochem Biophys Res Commun 437(1):67-73. [PubMed: 23791832]  [MGI Ref ID J:204417]

Samaco RC; Mandel-Brehm C; McGraw CM; Shaw CA; McGill BE; Zoghbi HY. 2012. Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome. Nat Genet 44(2):206-11. [PubMed: 22231481]  [MGI Ref ID J:181213]

Szulwach KE; Li X; Li Y; Song CX; Wu H; Dai Q; Irier H; Upadhyay AK; Gearing M; Levey AI; Vasanthakumar A; Godley LA; Chang Q; Cheng X; He C; Jin P. 2011. 5-hmC-mediated epigenetic dynamics during postnatal neurodevelopment and aging. Nat Neurosci 14(12):1607-16. [PubMed: 22037496]  [MGI Ref ID J:180327]

Wang IT; Reyes AR; Zhou Z. 2013. Neuronal morphology in MeCP2 mouse models is intrinsically variable and depends on age, cell type, and Mecp2 mutation. Neurobiol Dis 58C:3-12. [PubMed: 23659895]  [MGI Ref ID J:197982]

Health & husbandry

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

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryWhen maintained as a live colony, hemizygote female x wildtype (or FVB/NJ) male crosses may be used.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Noncarrier
   001800 FVB/NJ
 
  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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