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

Strain Name	Common Name	Molecular Mutation	Phenotype	Survival
B6.129P2(C)-Mecp2tm1.1Bird/J (003890)	MeCP2^Bird	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)	Mecp2^lox	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

Strain Name

Common Name

Molecular Mutation

Phenotype

Survival

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

(003890)

MeCP2^Bird

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)

Mecp2^lox

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

(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-Mecp2tm1Hzo/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-Mecp2tm1Bird/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

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