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Strain Name:

B6.129P2(C)-Mecp2tm1.1Bird/J

Stock Number:

003890

Availability:

Repository- Live


Price and Supply Information

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Genes & Alleles   Mecp2;   Mecp2tm1.1Bird;


Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Mating SystemHeterozygote x Inbred         (Female x Male)
Specieslaboratory mouse
Donating Investigator Adrian Bird,   University of Edinburgh
GenerationN4+17 (06-DEC-07)

Strain Description
Homozygous null mice are viable and appear normal at birth. No Mecp2 gene product (mRNA or protein) is detected in tissues. Mobility problems are apparent at 3-8 weeks of age. Mice exhibit hindlimb clasping and uneven breathing. An uneven wearing of teeth associated with misalignment of the jaws is observed in 50% of the animals. Adult males do not mate and their testes remain internal although sperm are present in the cauda epididymis. Symptom progression is variable, but mice can be expected to undergo weight loss, shivering, continued mobility problems before succumbing. Expected lifespan is about 50-60 days. Heterozygous female mice display mobility problems and hindlimb clasping starting at about 6 months, but the symptoms appear not to be progressive. This mutant mouse strain represents a model that may be useful in studies related to Rett Syndrome.

Importation of this model was supported in part by the Rett Syndrome Research Foundation.

Strain Development
A targeting vector designed to insert loxP sites around exons 3 and 4 was transfected into 129P2/OlaHsd-derived E14TG2a embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. Chimeric offspring were bred to C57BL/6 mice to produce heterozygous floxed females. The floxed line was bred to homozygosity and maintained in this form. Homozygous floxed female mice were crossed with male CMVCre mice (BALB/c background) in which the Cre gene is located on the X chromosome and is ubiquitously expressed. The resulting female mice, heterozygous for the recombined allele, were mated with wild type C57BL/6 animals. Each subsequent generation of heterozygous females have been mated to inbred C57BL/6 males to maintain the line.

Related Disease (OMIM) Terms

Rett Syndrome; RTT
Mammalian Phenotype Terms assigned by genotype

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

Mecp2tm1.1Bird/Mecp2+

        involves: 129P2/OlaHsd * C57BL/6
  • behavior/neurological phenotype
  • hypoactivity (MGI Ref ID J:67910)
    • although heterozygous females initially show no symptoms and raise normal litters, they acquire inertia at ages greater than 3 months
    • in an open field test, visit fewer squares, spend more time being immotile and rear less than controls, however this is not due to increased anxiety, as fecal bolus counts, grooming times and time spent in different zones of the field are similar to controls
  • limb grasping (MGI Ref ID J:67910)
    • acquire hindlimb clasping at ages greater than 3 months
  • respiratory system phenotype
  • abnormal breathing (MGI Ref ID J:67910)
    • often exhibit breathing irregularities by 9 months of age

Mecp2tm1.1Bird/Mecp2tm1.1Bird

        involves: 129P2/OlaHsd * C57BL/6
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:67910)
    • variable progression of symptoms leads to rapid weight loss and death at about 54 days of age
  • behavior/neurological phenotype
  • abnormal gait (MGI Ref ID J:67910)
    • develop a stiff, uncoordinated gait between 3 and 8 weeks of age
  • hypoactivity (MGI Ref ID J:67910)
    • exhibit reduced spontaneous movement between 3 and 8 weeks of age
  • limb grasping (MGI Ref ID J:67910)
    • most mutants develop hindlimb clasping after 7 weeks of age
  • growth/size phenotype
  • weight loss (MGI Ref ID J:67910)
    • variable progression of symptoms leads to rapid weight loss and death at about 54 days of age
  • respiratory system phenotype
  • abnormal breathing (MGI Ref ID J:67910)
    • most mutants exhibit irregular breathing after 3-8 weeks of age
  • craniofacial phenotype
  • abnormal jaw morphology (MGI Ref ID J:67910)
    • frequently exhibit misalignment of the jaws
  • abnormal tooth morphology (MGI Ref ID J:67910)
    • frequently exhibit uneven wearing of the teeth
  • skeleton phenotype
  • abnormal jaw morphology (MGI Ref ID J:67910)
    • frequently exhibit misalignment of the jaws
  • hearing/vestibular/ear phenotype
  • abnormal hearing physiology (MGI Ref ID J:67910)
    • some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected

Mecp2tm1.1Bird/Y

        involves: 129P2/OlaHsd * C57BL/6
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:67910)
    • variable progression of symptoms leads to rapid weight loss and death at about 54 days of age
  • behavior/neurological phenotype
  • abnormal gait (MGI Ref ID J:67910)
    • develop a stiff, uncoordinated gait between 3 and 8 weeks of age
  • hypoactivity (MGI Ref ID J:67910)
    • exhibit reduced spontaneous movement between 3 and 8 weeks of age
  • limb grasping (MGI Ref ID J:67910)
    • most mutants develop hindlimb clasping after 7 weeks of age
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:67910)
    • mutants mated to C57BL/6 (mixed 129P2/OlaHsd and C57BL/6 background) mice are substantially underweight from 4 weeks with full penatrance
    • weight loss (MGI Ref ID J:67910)
      • variable progression of symptoms leads to rapid weight loss and death at about 54 days of age
  • weight gain (MGI Ref ID J:67910)
    • mutants on the mixed 129P2/OlaHsd and C57BL/6 background mated to 129 mice are the same weight as controls until 8 weeks of age, when they gain weight and become heavier than controls with an increase in deposited fat
  • respiratory system phenotype
  • abnormal breathing (MGI Ref ID J:67910)
    • most mutants exhibit irregular breathing after 3-8 weeks of age
  • reproductive system phenotype
  • cryptorchism (MGI Ref ID J:67910)
    • testes of mutant males are always internal
  • craniofacial phenotype
  • abnormal jaw morphology (MGI Ref ID J:67910)
    • frequently exhibit misalignment of the jaws
  • abnormal tooth morphology (MGI Ref ID J:67910)
    • frequently exhibit uneven wearing of the teeth
  • endocrine/exocrine gland phenotype
  • cryptorchism (MGI Ref ID J:67910)
    • testes of mutant males are always internal
  • skeleton phenotype
  • abnormal jaw morphology (MGI Ref ID J:67910)
    • frequently exhibit misalignment of the jaws
  • hearing/vestibular/ear phenotype
  • abnormal hearing physiology (MGI Ref ID J:67910)
    • some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected

Gene & Allele Details

Allele Symbol Mecp2tm1.1Bird
Allele Name targeted mutation 1.1, Adrian Bird
Mutation Made By Adrian Bird,   University of Edinburgh
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14TG2a
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Mecp2, methyl CpG binding protein 2
Chromosome X
Gene Common Name(s) 1500041B07Rik; AUTSX3; BB130002; D630021H01Rik; DKFZp686A24160; MRX16; MRX79; MRXS13; MRXSL; Mbd5; PPMX; RIKEN cDNA 1500041B07 gene; RIKEN cDNA D630021H01 gene; RTS; RTT; WBP10; expressed sequence BB130002;
Molecular Note Insertion of a neomycin resistance cassette into the Mecp2 gene introduced loxP sites that flank exons 3 and 4, and added an intron and polyadenylation signal from the human beta globin gene. A CMV-Cre mediated recombination event in the germline then removed exons 3 and 4. Northern blot analysis did not detect Mecp2 mRNA in tissues of mutant male mice (-/y), nor did Western blot analysis detect protein in these tissues. [MGI Ref ID J:67910]

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for JAX® GEMM® Strains

Genotyping Protocols

Mecp2tm1.1Bird

Colony Maintenance

Breeding & HusbandryHeterozygous females breed best when under 6 months of age. Coat color expected from breeding:Black
Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying other alleles of Mecp2
005439   B6.129S-Mecp2tm1Hzo/J
006847   B6;129P2-Mecp2tm1Bird/J
006849   B6;129P2-Mecp2tm2Bird/J
View Strains carrying other alleles of Mecp2     (3 strains)

Additional Web Information

Congenic Nomenclature
JAX® NOTES, Summer 2002; 486. New Mouse Model for Rett Syndrome.

Animal Health Reports

Room Number           AX12

Research Applications

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

Mecp2tm1.1Bird related

Mouse/Human Gene Homologs
Rett syndrome

Neurobiology Research
Ataxia (Movement) Defects
Neurodevelopmental Defects (Rett's syndrome)

References

Selected Reference(s)

Guy J; Hendrich B; Holmes M; Martin JE; Bird A. 2001. A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome. Nat Genet 27(3):322-6. [PubMed: 11242117]  [MGI Ref ID J:67910]

Additional References

Price and Supply Information

Strain Name: B6.129P2(C)-Mecp2tm1.1Bird/J
Stock Number: 003890

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. The shipping destinations are:

*Pricing for Shipping Destination selected:

        USA, Canada and Mexico

Price(s) in US dollars ($)Genotype(s) Provided
Individual Mouse Price $198.60Heterozygous for Mecp2tm1.1BirdFemale
Individual Mouse Price $198.60Hemizygous for Mecp2tm1.1BirdMale
Pair $214.10Heterozygous for Mecp2tm1.1Bird x C57BL/6J (000664)

Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes Histology and Tissue Collection Services are available for all JAX® Mice strains. For more information, please contact Customer Service at orderquest@jax.org or 1-800-422-6423.
Hemizygotes are not available as a pair, only males are available.
Usually shipped between four and eight weeks of age.
This strain is included in the Induced Mutant Resource Colony collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.
LicensingSee General Terms and Conditions below for Licensing and Use Restrictions  
Control InformationView Control Information in Strain Details.
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General Terms and Conditions

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Effective September 26, 2007: License Requirements for Strains using Cre-lox Technology only apply in Canada, see Licenses for Strains using Cre-lox Technology.

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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