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Strain Name:

B6;129P2-Mecp2tm2Bird/J

Stock Number:

006849

Availability:

Repository- Live


General Terms and Conditions

Former Name      B6.129P2-Mecp2tm2Bird/J    (Changed: 06-MAY-08 )
Genes & Alleles   Mecp2;   Mecp2tm2Bird;


Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Targeted Mutation
Mating SystemHemizygote x +/+ sibling         (Female x Male)
Specieslaboratory mouse
Donating Investigator Adrian Bird,   University of Edinburgh
GenerationN6+N1 (14-NOV-07)

Strain Description
These mice possess a loxP-flanked STOP cassette in intron 2 of the targeted gene on the X chromosome. Western blot and hybridization analysis confirm the absence of wildtype protein from the targeted allele. Hemizygous (Mecp2lox-Stop/y) males do not breed and develop Rett syndrome symptoms (reduced mobility, hindlimb clasping) at approximately 6 weeks of age, with death occurring at approximately 11 weeks of age. Heterozygous females are fertile until developing Rett syndrome characteristics at 4-12 months of age. This Rett syndrome-like phenotype is similar to that observed for the traditional knock-out allele (see Stock No. 003890). Cre recombinase-mediated removal of the floxed-STOP cassette restores transcription from the targeted allele and MECP2 protein activity to normal, and reverses the Rett syndrome-like neurological defects.

This mutant mouse strain may be bred to a strain expressing tamoxifen inducible Cre recombinase in most tissues (see Stock No. 004682).

Mice with this X-linked lox-STOP mutation may be useful in neurological and developmental studies of Rett syndrome and its amelioration upon excision of the lox-STOP cassette.

Strain Development
A targeting vector was designed to insert a loxP-flanked STOP-Neo cassette into intron 2 of the targeted gene. The STOP-Neo cassette is composed of a PGK-Neo cassette followed by the 3' portion of the yeast His3 gene, an SV40 polyadenylation sequence, and a false translation initiation codon followed by a 5' splice donor site. The construct was electroporated into 129P2/OlaHsd-derived E14TG2a embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts, and chimeric offspring were bred to C57BL/6 mice. Heterozygous female mutants were bred to wildtype C57BL/6 males for 5 generations prior to arrival at The Jackson Laboratory. A SNP (single nucleotide polymorphism) panel analysis performed by The Jackson Laboratory revealed that this strain is on a mixed B6;129 genetic background background.

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.

Mecp2tm2Bird/Mecp2+

        involves: 129P2/OlaHsd * C57BL/6
  • nervous system phenotype
  • reduced long term potentiation (MGI Ref ID J:118365)
    • deficits in hippocampal long term potential are rescued by tamoxifen treatment

Mecp2tm2Bird/Y

        involves: 129P2/OlaHsd * C57BL/6
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:118365)
    • male mice survive only until 11 weeks
  • behavior/neurological phenotype
  • abnormal behavior (MGI Ref ID J:118365)
    • RTT-like symptoms (inertia, gait, hindlimb clasping, tremor, irregular breathing and poor general condition) show a progression of aggregated symptom scores during the last 4 weeks of life at 12 weeks mice display low stance, inertia, tremor, arrhythmic breathing, splayed himdlimb and moderate hindlimb clasping
    • limb grasping (MGI Ref ID J:118365)
      • at 12 weeks, mice display moderate hindlimb clasping
    • tremors (MGI Ref ID J:118365)
      • at 12 weeks
  • nervous system phenotype
  • abnormal nervous system physiology (MGI Ref ID J:118365)
    • mice develop neurological symptoms are 4 weeks
  • respiratory system phenotype
  • abnormal breathing (MGI Ref ID J:118365)
    • at 12 weeks

The following phenotype relates to a compound genotype created using this strain.
Contact JAX® Services jaxservices@jax.org for customized breeding options.

Mecp2tm2Bird/Mecp2+ Tg(CAG-cre/Esr1)5Amc/?

        involves: 129P2/OlaHsd * C57BL/6 * CBA   (conditional)
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:118365)
    • most mice die following tamoxifen injection
  • behavior/neurological phenotype
  • abnormal behavior (MGI Ref ID J:118365)
    • RTT-like symptoms (inertia, gait, hindlimb clasping, tremor, irregular breathing and poor general condition) show a progression of aggregated symptom scores during the last 4 weeks of life which are rescued by tamoxifen treatment
  • nervous system phenotype
  • abnormal nervous system physiology (MGI Ref ID J:118365)
    • some mice develop neurological problems following high dose tamoxifen injection
  • growth/size phenotype
  • obese (MGI Ref ID J:118365)

Gene & Allele Details

Allele Symbol Mecp2tm2Bird
Allele Name targeted mutation 2, Adrian Bird
Common Name(s) Mecp2lox-stop;
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 Cre recombinase-reversible gene inactivation was accomplished by insertion into intron 2 of a 3.1-kb DNA fragment containing a loxP-flanked "neostop" cassette, which comprises a neomycin resistance cassette followed by a transcriptional/translational "stop" cassette composed of 550 bp of 3' sequence from the Saccharomyces cerevisiae His3 gene, an SV40 polyadenylation sequence, and a synthetic sequence containing a false translation initiation codon (ATG) immediately followed by a consensus splice donor sequence. No protein product from the endogenous gene was detected by western blot or immunofluorescence analysis of brains of mutant mice. [MGI Ref ID J:111323] [MGI Ref ID J:118365] [MGI Ref ID J:75205]

Control Information

  Control
   Wild-type from the colony
   100492 B6129PF1/J (approximate)
 
  Considerations for Choosing Controls

Genotyping Protocols

Mecp2tm2Bird

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, females hemizygous for this X-linked mutation can be bred with wildtype siblings. The donating investigator recommends replacing hemizygous female breeders when Rett syndrome symptoms appear or when females fail to produce or care for regular litters (may be as early as 4-6 months. The donating investigator also reports that breeding performance may be improved if mice are maintained on a mixed C57BL/6;BALB/c background.
Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying other alleles of Mecp2
003890   B6.129P2(C)-Mecp2tm1.1Bird/J
005439   B6.129S-Mecp2tm1Hzo/J
006847   B6;129P2-Mecp2tm1Bird/J
View Strains carrying other alleles of Mecp2     (3 strains)

Additional Web Information

Congenic Nomenclature
Cre-lox or FLP-FRT Systems

Animal Health Reports

Room Number           AX11

Research Applications

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

Mouse/Human Gene Homologs
Rett syndrome

Neurobiology Research
Behavioral and Learning Defects
Cre-lox System (loxP-flanked Sequences)
Neurodevelopmental Defects (Rett's syndrome)

Research Tools
Cre-lox System (loxP-flanked Sequences)
Developmental Biology Research (Cre-lox System)

Mecp2tm2Bird related

Neurobiology Research
Ataxia (Movement) Defects

References

Selected Reference(s)

Guy J; Gan J; Selfridge J; Cobb S; Bird A. 2007. Reversal of neurological defects in a mouse model of Rett syndrome. Science 315(5815):1143-7. [PubMed: 17289941]  [MGI Ref ID J:118365]

Additional References

Price and Supply Information

Strain Name: B6;129P2-Mecp2tm2Bird/J
Stock Number: 006849

Price Details

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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 Usually shipped between four and eight weeks of age.
This strain is included in the Induced Mutant Resource Colony collection.
LicensingSee General Terms and Conditions below for Licensing and Use Restrictions  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

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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