Rett Syndrome Mouse Model Resource

Explore our growing collection of mouse models of Rett Syndrome, use this resource to quickly identify the stain you need for your research.

Comparison of featured Rett Syndrome models

This table summarizes the differences between important mouse models for Rett Syndrome to help you easily find the right strain for your research.

Strain Name

Common Name

Molecular Mutation

Phenotype

Survival

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

003890

MeCP2Bird Null (knockout) mutation of methy CpG binding protein 2 (Mecp2) deleting exons 3 and 4 Null mice (homozygous females and hemizygous males):
  • exhibit abnormal gait and hypoactivity at 3 and 8 weeks 
  • develop hindlimb clasping after 7 weeks of age 
Heterozygous females:
  • 33% decrease in neuron number by 7-9 weeks 
  • develop hindlimb clasping after 3 months of age
Null mice die by approximately 54 days of age

B6.129S-Mecp2tm1Hzo/J

005439

Mecp2308 Knockout mutation of methy CpG binding protein 2 (Mecp2) where a stop codon was inserted downstream to codon 308 in exon 4 allowing translation of the methyl-CpG binding domain and the transcriptional repression domain Hemizygous males: 
  • show impaired coordination, abnormal locomotor behavior, hypoactivity and develop subtle tremors at around 6 weeks of age that worsen with age and are visibly apparent by 4 months of age
  • seizures observed in some mice after 8 months of age 
Heterozygous females show impaired coordination:
  •  69% display rapid and repetitive movement of the forelimbs 
  • 62% exhibit tremors
Hemizygous males show 10% premature death between 10 and 12 months of age

B6.129P2-Mecp2tm1Bird/J

007177

Mecp2lox Floxed allele where loxP sites flank exons 3 and 4 and has an intron and polyadenylation signal from human beta globin Homozygous floxed mice are normal, when bred to strains expressing cre in the nervous system, mice develop Rett Syndrome phenotypes Normal life span without cre expression

Featured JAX® Mice Models of Rett Syndrome

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

003890

  • Common name: MeCP2Bird
  • Genetic background: C57BL/6, incipient congenic (N5)
  • Carries a null (knockout) mutation of methy CpG binding protein 2 (Mecp2) deleting exons 3 and 4 (Guy et al., 2001)
  • Homozygote females and hemizygote males have abnormal gait and hypoactivity at 3 and 8 weeks of age, develop hindlimb clasping after 7 weeks of age
  • Heterozygote females have 33% decrease in neuron number by 7-9 weeks, and acquire hindlimb clasping after 3 months of age
  • Rapid weight loss and death in null mice by 54 days of age

B6.129S-Mecp2tm1Hzo/J 

005439

  • Common name: Mecp2308
  • Genetic background: C57BL/6J (000664), fully congenic (N14)
  • Carries a knockout  mutation of methy CpG binding protein 2 (Mecp2) in which a stop codon is inserted downstream to codon 308 (exon 4) allowing translation of the methyl-CpG binding domain and the transcriptional repression domain (Shahbazian et al., 2002)
  • By 6 weeks of age, male hemizygotes exhibit tremors, progressive motor dysfunction, oily, disheveled fur, hypoactivity, myoclonic seizures, and kyphosis
  • 62% of heterozygous females exhibit tremors
  • Approxmately 90% of hemizygous males survive to 12 months

B6.129P2-Mecp2tm1Bird/J

007177

  • Common name: Mecp2lox
  • Genetic background: C57BL/6, incipient congenic (N5)
  • Carries a floxed allele with loxP sites flanking exons 3 and 4 and an added intron and polyadenylation signal from human beta globin (Guy et al., 2001)
  • Prior to breeding with a cre expressing strain, homozygotes are normal in phenotype
  • Following breeding to a strain expressing cre in the central and peripheral nervous system (003771), mice develop a neurological phenotype that mimics Rett syndrome
  • Following breeding to a strain expressing cre in GABAergic neurons (017535), mice exhibit behaviors common to those seen in Rett Syndrome and Autism Spectrum Disorders