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 of Featured Rett Syndrome Models

Featured JAX® Mice Models of Rett Syndrome

B6.129P2(C)-Mecp2^tm1.1Bird/J

(003890)

• Common name: MeCP2^Bird
• 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-Mecp2^tm1Hzo/J

(005439)

• 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-Mecp2^tm1Bird/J

(007177)

• Common name: Mecp2^lox
• 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