Description

Strain Information

Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Heterozygote x Homozygote         (Female x Male)   29-AUG-09 Species laboratory mouse Generation N5F1 (18-AUG-09)   Donating Investigator Adrian Bird,   University of Edinburgh

Description
These mice possess two functional loxP sites flanking exons 3-4 of the targeted gene on the X chromosome (the donating investigator reports that the middle loxP site is non-functional). Homozygous females and hemizygous males are viable and fertile. Northern blot analysis showed the expected mature transcript from the Mecp2^lox locus as well as a transcript in which the beta-globin intron was unspliced. When these mutant mice are bred to mice that express Cre recombinase, resulting offspring will have exons 3-4 deleted in the cre-expressing tissue(s). Mice with this X-linked floxed mutation may be useful in neurological and developmental studies of Rett syndrome.

For example, when crossed to a strain expressing Cre recombinase in nervous tissue (see Stock No. 003771), this mutant mouse strain develops a neurological phenotype that mimics Rett syndrome.

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.

Control Information

	Control
	000664 C57BL/6J
Considerations for Choosing Controls

Related Strains

Strains carrying   Mecp2^tm1Bird allele

006847

B6;129P2-Mecp2tm1Bird/J

View Strains carrying   Mecp2^tm1Bird     (1 strain)

Strains carrying other alleles of Mecp2

003890	B6.129P2(C)-Mecp2tm1.1Bird/J
006849	B6.129P2-Mecp2tm2Bird/J
005439	B6.129S-Mecp2tm1Hzo/J

View Strains carrying other alleles of Mecp2     (3 strains)

Additional Web Information

Introduction to Cre-lox technology

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms

Rett Syndrome; RTT - Models with phenotypic similarity to human disease where etiologies involve orthologs.1

1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

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

Mecp2^tm1Bird/Y

        (129S6.129P2-Mecp2^tm1Bird x C57BL/6)F1

• life span-post-weaning/aging
• *normal* life span-post-weaning/aging (MGI Ref ID J:135825)
  • no early mortality is observed in contrast to Mecp2-null animals
• growth/size phenotype
• increased body weight (MGI Ref ID J:135825)
  • a mild increase (animals are about 1 gram heavier) is observed relative to wild-type or F1 mutants from an FVB/N cross
• behavior/neurological phenotype
• abnormal nest building behavior (MGI Ref ID J:135825)
  • mice show deficits in nest-building relative to wild-type, with fewer animals building or completing nests than wild-type animals over the same time period
• abnormal social investigation (MGI Ref ID J:135825)
  • mice have altered social behavior and spend more time than wild-type animals interacting with an unfamiliar mouse or with a 'familiar' mouse that has been reintroduced into the cage
• abnormal touch/ nociception (MGI Ref ID J:135825)
  • mutants exhibit deficit in pain recognition rather than significant defect in pain sensitivity
• increased thermal nociceptive threshold (MGI Ref ID J:135825)
  • mice show increased latency in the hot plate assay
• decreased startle reflex (MGI Ref ID J:135825)
  • mice have a decreased startle response relative to wild-type
• impaired coordination (MGI Ref ID J:135825)
  • mice display impaired performance in coordination tasks like the rotating rod, hanging wire, and dowel walking tests
• nervous system phenotype
• decreased prepulse inhibition (MGI Ref ID J:135825)
  • mice show decreased prepulse inhibition at 74 and 82 decibels compared to wild-type
• touch/vibrissae phenotype
• abnormal touch/ nociception (MGI Ref ID J:135825)
  • mutants exhibit deficit in pain recognition rather than significant defect in pain sensitivity
• increased thermal nociceptive threshold (MGI Ref ID J:135825)
  • mice show increased latency in the hot plate assay
• hearing/vestibular/ear phenotype
• decreased startle reflex (MGI Ref ID J:135825)
  • mice have a decreased startle response relative to wild-type

Mecp2^tm1Bird/Y

        (129S6.129P2-Mecp2^tm1Bird x FVB/N)F1

• behavior/neurological phenotype
• *normal* behavior/neurological phenotype (MGI Ref ID J:135825)
  • no overt abnormalities are observed; mutants do not exhibit impaired coordination in a variety of tests measured
  • mice do not show overt abnormalities like tremor or limb grasping
• abnormal contextual conditioning behavior (MGI Ref ID J:135825)
  • percentage of time spent freezing is elevated relative to wild-type when placed back into the conditioning chamber
• abnormal cued conditioning behavior (MGI Ref ID J:135825)
  • percentage of time spent freezing is elevated relative to wild-type when re-exposure to a previously experienced cue
• abnormal social investigation (MGI Ref ID J:135825)
  • mice have altered social behavior and spend more time than wild-type animals interacting with an unfamiliar mouse or with a 'familiar' mouse that has been reintroduced into the cage
• abnormal touch/ nociception (MGI Ref ID J:135825)
  • mutants exhibit deficit in pain recognition rather than significant defect in pain sensitivity
• decreased anxiety-related response (MGI Ref ID J:135825)
  • mice show decrease anxiety-related behavior in open field assays than wild-type mice
• increased vertical activity (MGI Ref ID J:135825)
  • increased number of vertical explorations is observed compared to wild-type; this is indicative of reduced anxiety
• life span-post-weaning/aging
• *normal* life span-post-weaning/aging (MGI Ref ID J:135825)
  • no early mortality is observed in contrast to Mecp2-null animals
• nervous system phenotype
• decreased prepulse inhibition (MGI Ref ID J:135825)
  • mice show decreased prepulse inhibition compared to wild-type
• respiratory system phenotype
• abnormal breathing (MGI Ref ID J:135825)
  • at 4 months, respiratory pattern is qualitatively different than in wild-type; coefficient of variability of the respiratory rhythm is higher than in wild-type
• apnea (MGI Ref ID J:135825)
  • increase in apnea incidence (39.5/hour) is observed compared to wild-type (5.8/hour)
• touch/vibrissae phenotype
• abnormal touch/ nociception (MGI Ref ID J:135825)
  • mutants exhibit deficit in pain recognition rather than significant defect in pain sensitivity
• growth/size phenotype
• *normal* growth/size phenotype (MGI Ref ID J:135825)
  • no size difference is detected relative to wild-type, unlike mice on the C57BL/6 background

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

Mecp2^tm1Bird/Y Tg(Nes-cre)1Kln/0

        involves: 129P2/OlaHsd * C57BL/6 * SJL   (conditional)

• life span-post-weaning/aging
• premature death (MGI Ref ID J:67910)
• behavior/neurological phenotype
• abnormal gait (MGI Ref ID J:67910)
  • develop a stiff, uncoordinated gait
• hypoactivity (MGI Ref ID J:67910)
• limb grasping (MGI Ref ID J:67910)
• craniofacial phenotype
• 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)
• growth/size phenotype
• decreased body weight (MGI Ref ID J:67910)
• reproductive system phenotype
• cryptorchism (MGI Ref ID J:67910)

View Research Applications

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
Neurodevelopmental Defects
      Rett's syndrome

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

Mecp2^tm1Bird related

Neurobiology Research
Ataxia (Movement) Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol		Mecp2tm1Bird
Allele Name		targeted mutation 1, Adrian Bird
Allele Type		Targeted (Floxed/Frt)
Common Name(s)		Mecp2lox;
Mutation Made By		Adrian Bird,   University of Edinburgh
Strain of Origin		129P2/OlaHsd
ES Cell Line Name		E14TG2a
ES Cell Line Strain		129P2/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; RS; RTS; RTT; WBP10; expressed sequence BB130002;
Molecular Note		Insertion of a neomycin resistance cassette into the gene introduced loxP sites that flank exons 3 and 4, and added an intron and polyadenylation signal from the human beta globin gene. From the mutated allele, Northern blot analysis detected the wild type mature transcript and also a transcript in which the beta globin intron was unspliced. [MGI Ref ID J:67910]

Genotyping

Genotyping Information

Genotyping Protocols

Mecp2^tm1Bird, Standard PCR

Helpful Links

Genotyping resources and troubleshooting

References

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

Mecp2^tm1Bird related

Fyffe SL; Neul JL; Samaco RC; Chao HT; Ben-Shachar S; Moretti P; McGill BE; Goulding EH; Sullivan E; Tecott LH; Zoghbi HY. 2008. Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress. Neuron 59(6):947-58. [PubMed: 18817733]  [MGI Ref ID J:143360]

Peddada S; Yasui DH; LaSalle JM. 2006. Inhibitors of differentiation (ID1, ID2, ID3 and ID4) genes are neuronal targets of MeCP2 that are elevated in Rett syndrome. Hum Mol Genet 15(12):2003-14. [PubMed: 16682435]  [MGI Ref ID J:112064]

Samaco RC; Fryer JD; Ren J; Fyffe S; Chao HT; Sun Y; Greer JJ; Zoghbi HY; Neul JL. 2008. A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome. Hum Mol Genet 17(12):1718-27. [PubMed: 18321864]  [MGI Ref ID J:135825]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX11

Colony Maintenance

Breeding & Husbandry	Mutant mice were bred to C57BL/6J mice to generate this congenic strain. When maintaining the live congenic colony, females homozygous for this X-linked mutation can be bred with males hemizygous for this X-linked mutation.
Mating System	Heterozygote x Homozygote         (Female x Male)   29-AUG-09
Diet Information	LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply

Repository-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 approximately 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 two business days following order placement.

Supply Notes

Usually shipped between four and eight weeks of age. This strain is included in the Induced Mutant Resource Colony collection.

Control Information

	Control
	000664 C57BL/6J
Considerations for Choosing Controls
USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
International - 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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fax:	207-288-6655

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