Rett Syndrome Mouse Model Resource
Explore our growing collection of mouse models of Rett Syndrome, use this resource to quickly identify the best strain for your research.
- See a complete list of JAX® Mice for Rett Syndrome
- Learn about Rett Syndrome Efficacy Studies
Comparison table of featured Rett Syndrome models
|
Strain Name |
Common Name |
Molecular Mutation |
Phenotype |
Survival |
|---|---|---|---|---|
|
003890 |
MeCP2Bird | Null (knockout) mutation of methy CpG binding protein 2 (Mecp2) deleting exons 3 and 4 | Null mice (homozygous females and hemizygous males):
|
Null mice die by approximately 54 days of age |
|
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:
|
Hemizygous males show 10% premature death between 10 and 12 months of age |
|
007177 |
Mecp2lox | Floxed allele where loxP sites flank exons 3 and 4 | 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
- Common name: MeCP2Bird
- Genetic background: C57BL/6J congenic (>N30)
- Carries a null (knockout) mutation of methyl CpG binding protein 2 (Mecp2) deleting exons 3 and 4 (Guy et al., 2001)
- Homozygous females and hemizygous males have abnormal gait and hypoactivity at 3 and 8 weeks of age, develop hindlimb clasping after 7 weeks of age
- Heterozygous females have 33% decrease in neuron number by 7-9 weeks, and acquire hindlimb clasping after 3 months of age
- Rapid weight loss is observed resulting in death in null mice by about 54 days of age
- Heterozygous Mecp2 females are bred to C57BL/6J males every generation
B6.129S-Mecp2tm1Hzo/J
- Common name: Mecp2308
- Genetic background: C57BL/6J (000664), fully congenic (N14)
- Carries a targeted 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
- Approximately 90% of hemizygous males survive to 12 months
B6.129P2-Mecp2tm1Bird/J
- 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 the central and peripheral nervous system (e.g. Nes-cre, 003771), mice develop a neurological phenotype that mimics Rett Syndrome
- Following breeding to a strain expressing cre in GABAergic neurons (e.g. Slc32a1-cre,017535), mice exhibit behaviors common to those seen in Rett Syndrome and Autism Spectrum Disorders