JAX
Mouse Strain Datasheet - 003890
B6.129P2(C)-Mecp2tm1.1Bird/J
Also Known As: Mecp2-
This Mecp2 knockout strain is a mouse model of human Rett Syndrome. Mecp2-deficient males (Mecp2-/Y) exhibit mobility problems and a range of Rett Syndrome-like characteristics at 3-8 weeks of age. Heterozygous females exhibit a much later onset (~6 months of age).
Donating Investigator
Adrian Bird, University of Edinburgh
Genetic overview
| Genetic Background | Generation |
|---|---|
|
N4+N40
|
Mecp2tm1.1Bird
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout) | Mecp2 | methyl CpG binding protein 2 |
Base Price
Starting at:
| $205.90 Domestic price for female 4-week |
| $230.62 Domestic price for breeder pair |
Detailed Description
The Mecp2 knockout mutation is X-linked; therefore the Mecp2-deficient mice are Mecp2-/- (homozygous) females and Mecp2-/Y (hemizygous) males.
Mecp2 null mice are viable and appear normal at birth. No Mecp2 gene product (mRNA or protein) is detected in tissues. Mobility problems are apparent at 3-8 weeks of age. Mice exhibit hindlimb clasping and uneven breathing. An uneven wearing of teeth associated with misalignment of the jaws is observed in 50% of the animals. Adult males do not mate and their testes remain internal although sperm are present in the cauda epididymis. Symptom progression is variable, but mice can be expected to undergo weight loss, shivering, continued mobility problems before succumbing. Expected lifespan is about 50-60 days.
Heterozygous female mice display mobility problems and hindlimb clasping starting at about 6 months, but the symptoms appear not to be progressive.
This Mecp2 knockout strain is useful for studying Rett Syndrome.
Importation of this model was supported in part by the Rett Syndrome Research Foundation.
Development
A targeting vector was designed to insert loxP sites around exons 3 and 4 of the methyl CpG binding protein 2 gene (Mecp2) on the X chromosome. The construct was transfected into 129P2/OlaHsd-derived E14TG2a embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. Chimeric offspring were bred to C57BL/6 mice to produce heterozygous floxed females. The floxed line was bred to homozygosity and maintained in this form. Homozygous floxed female mice were crossed with male CMV-Cre mice (BALB/c background) in which the Cre gene is located on the X chromosome and is ubiquitously expressed. The resulting female mice, heterozygous for the germline recombined null allele, were mated with wild type C57BL/6 animals. Each subsequent generation of heterozygous females have been mated to inbred C57BL/6 males to maintain the line.
Control Suggestions
- Wild-type from the colony
- 000664 C57BL/6J
Additional Information
Selected References
- 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: 11242117MGI: J:67910
- Samaco RC; McGraw CM; Ward CS; Sun Y; Neul JL; Zoghbi HY. 2013. Female Mecp2+/- mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies. Hum Mol Genet 22(1):96-109. PubMed: 23026749MGI: J:189576
Mecp2tm1.1Bird
Allele Symbol: Mecp2tm1.1Bird
| Allele Name | targeted mutation 1.1, Adrian Bird |
|---|---|
| Allele Type | Targeted (Null/Knockout) |
| Allele Synonym(s) | MeCP2Bird; Mecp2-; Mecp2tm1+1Bird; Mecp2tm1-1Bird |
| Gene Symbol and Name | Mecp2, methyl CpG binding protein 2 |
| Gene Synonym(s) | 1500041B07Rik; 1500041B07Rik; AUTSX3; BB130002; D630021H01Rik; D630021H01Rik; MRX16; MRX79; MRXS13; MRXSL; Mbd5; PPMX; RIKEN cDNA 1500041B07 gene; RIKEN cDNA D630021H01 gene; RS; RTS; RTT; WBP10; expressed sequence BB130002 |
| Strain of Origin | 129P2/OlaHsd |
| Chromosome | X |
| Molecular Note | Insertion of a neomycin resistance cassette into the Mecp2 gene introduced loxP sites that flank exons 3 and 4, and added an intron and polyadenylation signal from the human beta globin gene. A CMV-Cre mediated recombination event in the germline then removed exons 3 and 4. Northern blot analysis did not detect Mecp2 mRNA in tissues of mutant male mice (-/y), nor did Western blot analysis detect protein in these tissues. |
| Mutations Made By | Adrian Bird, University of Edinburgh |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
Genotype: Mecp2tm1.1Bird related
- Neurobiology Research
- Ataxia (Movement) Defects
- Neurodevelopmental Defects
- Rett's syndrome
Mammalian Phenotype Terms by Genotype
Genotype: Mecp2tm1.1Bird/Y
B6.129P2(C)-Mecp2tm1.1Bird/J
behavior/neurological phenotype
- hypoactivity
- mice exhibit decreased activity levels during the light cycle but not the dark cycle (MGI Ref ID J:241788)
cardiovascular system phenotype
- atrioventricular block
- atrioventricular block is seen at a higher rate in 2 month old males (MGI Ref ID J:241788)
- decreased heart rate
- increased heart rate variability
- (MGI Ref ID J:241788)
- irregular heartbeat
- 2 month old males present with a variety of spontaneous cardiac arrhythmias ranging from sinus pauses and atrioventricular block to aberrant ventricular contractions (MGI Ref ID J:241788)
- 2-3 month old males exhibit erratic heart rate patterns, with sudden pauses in the regular rhythm of the heart rate and bradycardic events (MGI Ref ID J:241788)
- treatment with atropine results in a drop in the frequency of arrhythmias (MGI Ref ID J:241788)
- treatment with both atropine and propranolol decreases the rate of sinus pauses but has little effect on the overall heart rate (MGI Ref ID J:241788)
- treatment with isoproterenol decreases the rate of sinus pauses (MGI Ref ID J:241788)
- treatment with propranolol does not decrease heart rate but increases sinus pauses (MGI Ref ID J:241788)
- treatment with propranolol or carbachol does not reduce the rate of sinus pauses (MGI Ref ID J:241788)
- ventricular premature beat
- 2 month old males are susceptible to premature ventricular contractions (MGI Ref ID J:241788)
homeostasis/metabolism phenotype
- decreased body temperature
- mice exhibit decreased temperature during both light and dark cycles (MGI Ref ID J:241788)
nervous system phenotype
- abnormal parasympathetic nervous system physiology
- mice are sensitive to parasympathetic blockade with atropine but have a blunted response to parasympathetic activation with carbachol, indicating excessive parasympathetic tone (MGI Ref ID J:241788)
- abnormal sympathetic nervous system physiology
- mice have a blunted response to either sympathetic blockade with propranolol or activation with isoproterenol (MGI Ref ID J:241788)
The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain
Genotype: Mecp2tm1.1Bird/Mecp2+
involves: 129P2/OlaHsd
nervous system phenotype
- abnormal cerebral cortex morphology
- decrease in the number of ATRX containing foci in null cells only (MGI Ref ID J:194039)
- abnormal hippocampus morphology
- decrease in the number of ATRX containing foci in null cells only (MGI Ref ID J:194039)
- decreased neuron number
- 7-9 week old adults have 33% the number of Mecp2-expressing neurons compared to young adult wild-type (MGI Ref ID J:108953)
- compared to age-matched wild-type adults, 24-95 week old heterozygotes have 68% the number of Mecp2-expressing cortical neurons; number of Mecp2-expressing neurons is significantly higher in younger mutants than in older mutants (MGI Ref ID J:108953)
cellular phenotype
- abnormal dosage compensation, by inactivation of X chromosome
- X-chromosome inactivation becomes unbalance in older mutants, with a larger percentage expressing Mecp2 compared to younger mutants (>50%) (MGI Ref ID J:108953)
Genotype: Mecp2tm1.1Bird/Mecp2+
involves: 129P2/OlaHsd * C57BL/6
behavior/neurological phenotype
- hypoactivity
- although heterozygous females initially show no symptoms and raise normal litters, they acquire inertia at ages greater than 3 months (MGI Ref ID J:67910)
- in an open field test, visit fewer squares, spend more time being immotile and rear less than controls, however this is not due to increased anxiety, as fecal bolus counts, grooming times and time spent in different zones of the field are similar to controlsols (MGI Ref ID J:67910)
- limb grasping
- acquire hindlimb clasping at ages greater than 3 months (MGI Ref ID J:67910)
respiratory system phenotype
- abnormal breathing pattern
- often exhibit breathing irregularities by 9 months of age (MGI Ref ID J:67910)
Genotype: Mecp2tm1.1Bird/Mecp2tm1.1Bird
involves: 129P2/OlaHsd * C57BL/6
mortality/aging
- premature death
- variable progression of symptoms leads to rapid weight loss and death at about 54 days of age (MGI Ref ID J:67910)
behavior/neurological phenotype
- abnormal gait
- develop a stiff, uncoordinated gait between 3 and 8 weeks of age (MGI Ref ID J:67910)
- hypoactivity
- exhibit reduced spontaneous movement between 3 and 8 weeks of age (MGI Ref ID J:67910)
- limb grasping
- most mutants develop hindlimb clasping after 7 weeks of age (MGI Ref ID J:67910)
growth/size/body region phenotype
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
- weight loss
- variable progression of symptoms leads to rapid weight loss and death at about 54 days of age (MGI Ref ID J:67910)
respiratory system phenotype
- abnormal breathing pattern
- most mutants exhibit irregular breathing after 3-8 weeks of age (MGI Ref ID J:67910)
craniofacial phenotype
- abnormal jaw morphology
- frequently exhibit misalignment of the jaws (MGI Ref ID J:67910)
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
skeleton phenotype
- abnormal jaw morphology
- frequently exhibit misalignment of the jaws (MGI Ref ID J:67910)
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
hearing/vestibular/ear phenotype
- abnormal hearing physiology
- some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected (MGI Ref ID J:67910)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd
nervous system phenotype
- abnormal excitatory postsynaptic currents
- evoked EPSC amplitudes show a 46% reduction compared to wild-type (MGI Ref ID J:126964)
- abnormal hippocampus physiology
- at P40, hippocampal NOVA1 (neuro-oncological ventral antigen 1) mRNA and protein levels are significantly upregulated relative to those in wild-type controls (MGI Ref ID J:235553)
- abnormal miniature excitatory postsynaptic currents
- mEPSCs show a significant decrease in frequency compared to wild-type (MGI Ref ID J:126964)
- abnormal nervous system physiology
- neurospecific isoforms of lysine (K)-specific demethylase 1A (KDM1A, also known as LSD1) are significantly upregulated in the hippocampus, cerebellum and cortex, along with a corresponding decrease in the ubiquitous KDM1A isoforms, suggesting that NOVA1-mediated upregulation of neurospecific KDM1A isoforms could be causally linked to hyperexcitability (MGI Ref ID J:235553)
- abnormal synaptic vesicle number
- neurons exhibit a 41% reduction in RRP charge relative to wild-type (MGI Ref ID J:126964)
- decreased CNS synapse formation
- nervous system phenotype
- no alteration in synaptic release probability is detected in neurons (MGI Ref ID J:126964)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * C57BL/6 * CBA
growth/size/body region phenotype
- increased body weight
- obese
- male mice become obese with increased weight of fat pads by 14-17 weeks (MGI Ref ID J:135824)
behavior/neurological phenotype
- abnormal motor coordination/balance
- (MGI Ref ID J:135824)
- abnormal social investigation
- mutants spend less time in chamber side containing familiar mouse than in side with a stranger relative to controls, although response to introduction of stranger is similar (MGI Ref ID J:135824)
- behavior/neurological phenotype
- ability to grip a wire and remain suspended for 1 minute is normal in mutants (MGI Ref ID J:135824)
- anxiety-related behaviors are similar to wild-type mice (MGI Ref ID J:135824)
- olfaction and motivation to eat were normal in mutants (MGI Ref ID J:135824)
- visual placing reflex and response to a soft touch to the side of the face are normal relative to wild-type (MGI Ref ID J:135824)
- limb grasping
- subtle clasping is observed starting at 6 weeks of age (MGI Ref ID J:135824)
adipose tissue phenotype
- increased total fat pad weight
- fat pads in 14-17 week old male mice weigh 2-3 times more than wild-type (MGI Ref ID J:135824)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * 129S6/SvEvTac
mortality/aging
- premature death
- however, treatment with fluvastatin improves lifespan (MGI Ref ID J:198551)
behavior/neurological phenotype
- decreased startle reflex
- even in mice treated with fluvastatin (MGI Ref ID J:198551)
- hypoactivity
- however, treatment with fluvastatin or lovastatin improves open-field activity (MGI Ref ID J:198551)
- impaired coordination
- however, treatment with fluvastatin or lovastatin improves coordination (MGI Ref ID J:198551)
- lethargy
- (MGI Ref ID J:198551)
- limb grasping
- (MGI Ref ID J:198551)
- tremors
- (MGI Ref ID J:198551)
homeostasis/metabolism phenotype
- increased brain cholesterol level
- at P56, but not P70 (MGI Ref ID J:198551)
- increased circulating LDL cholesterol level
- at P56 (MGI Ref ID J:198551)
- increased circulating cholesterol level
- increased circulating triglyceride level
- at P56 (MGI Ref ID J:198551)
- increased liver triglyceride level
craniofacial phenotype
- malocclusion
- (MGI Ref ID J:198551)
growth/size/body region phenotype
- decreased body weight
- (MGI Ref ID J:198551)
- malocclusion
- (MGI Ref ID J:198551)
liver/biliary system phenotype
- increased liver triglyceride level
respiratory system phenotype
- increased pulmonary respiratory rate
- even in mice treated with fluvastatin (MGI Ref ID J:198551)
skeleton phenotype
- malocclusion
- (MGI Ref ID J:198551)
nervous system phenotype
- increased brain cholesterol level
- at P56, but not P70 (MGI Ref ID J:198551)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * FVB
mortality/aging
- premature death
- median lifespan of 76 days (MGI Ref ID J:194039)
growth/size/body region phenotype
- increased body weight
- starting at 6 weeks of age and reaching a maximum weight by 8 weeks of age (MGI Ref ID J:194039)
nervous system phenotype
- abnormal brain development
- brain weight peaks at 7 weeks (1 week earlier than in controls) then declines (MGI Ref ID J:194039)
- abnormal cerebral cortex morphology
- decrease in the number of ATRX containing foci at 7 weeks of age and only a few foci are detected at 9 weeks of age (MGI Ref ID J:194039)
- abnormal hippocampus morphology
- decrease in the number of ATRX containing foci at 7 weeks of age and only a few foci are detected at 9 weeks of age (MGI Ref ID J:194039)
- decreased brain weight
- brain weight peaks at 7 weeks (1 week earlier than in controls) then declines (MGI Ref ID J:194039)
behavior/neurological phenotype
- abnormal gait
- at all ages tested and severity increases with age (MGI Ref ID J:194039)
- tremors
- at all ages tested and severity increases with age (MGI Ref ID J:194039)
integument phenotype
- disheveled coat
- at 8 weeks of age (MGI Ref ID J:194039)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * C57BL/6
mortality/aging
- premature death
- variable progression of symptoms leads to rapid weight loss and death at about 54 days of age (MGI Ref ID J:67910)
homeostasis/metabolism phenotype
- abnormal noradrenaline level
- 36% reduction in levels of norepinephrine within the vestibular nuclei (MGI Ref ID J:165306)
- males exhibit a 31%, 30%, and 61% decrease in norepinephrine in the prefrontal cortex at 3 weeks, the motor cortex at 3 weeks, and the cerebellum at 8 weeks of age (MGI Ref ID J:165306)
- mutants do not exhibit an increase in norepinephrine in the hippocampus from 3 to 8 weeks of age as seen in wild-type mice (MGI Ref ID J:165306)
- decreased serotonin level
- 5-HT levels are decreased in the hippocampus at 8 weeks of age but not at 3 weeks (MGI Ref ID J:165306)
- 5-HT turnover is increased in the prefrontal cortex and motor cortex (MGI Ref ID J:165306)
- males exhibit a 34% and 55% decrease in the 5-HT precursor, 5-HIAA in the prefrontal cortex at 3 weeks and the cerebellum at 8 weeks of age, respectively (MGI Ref ID J:165306)
- males exhibit a 36%, 30%, and 55% decrease in 5-HT in the prefrontal cortex at 3 weeks, the motor cortex at 3 weeks, and the cerebellum at 8 weeks of age, respectively (MGI Ref ID J:165306)
nervous system phenotype
- abnormal neurotransmitter level
behavior/neurological phenotype
- abnormal gait
- abnormal locomotor activation
- at 8 weeks of age, males show increased latency to start moving and to reach the wall of the open field apparatus (MGI Ref ID J:165306)
- abnormal locomotor coordination
- in the gait onset test, males take longer to exit a circle at 3 and 8 weeks of age (MGI Ref ID J:165306)
- ataxia
- males exhibit a greater front-base width overall and a larger hind-base width than wild-type mice by 3 weeks of age, a sign of ataxia (MGI Ref ID J:165306)
- hypoactivity
- exhibit reduced spontaneous movement between 3 and 8 weeks of age (MGI Ref ID J:67910)
- limb grasping
- most mutants develop hindlimb clasping after 7 weeks of age (MGI Ref ID J:67910)
- short stride length
- stride length is shorter than in wild-type mice at 8 weeks of age but not at 3 weeks of age (MGI Ref ID J:165306)
growth/size/body region phenotype
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
- decreased body weight
- Background Sensitivity - mutants mated to C57BL/6 (mixed 129P2/OlaHsd and C57BL/6 background) mice are substantially underweight from 4 weeks with full penatrance (MGI Ref ID J:67910)
- increased body weight
- Background Sensitivity - mutants on the mixed 129P2/OlaHsd and C57BL/6 background mated to 129 mice are the same weight as controls until 8 weeks of age, when they gain weight and become heavier than controls with an increase in deposited fat (MGI Ref ID J:67910)
- weight loss
- variable progression of symptoms leads to rapid weight loss and death at about 54 days of age (MGI Ref ID J:67910)
respiratory system phenotype
- abnormal breathing pattern
- most mutants exhibit irregular breathing after 3-8 weeks of age (MGI Ref ID J:67910)
reproductive system phenotype
- cryptorchism
- testes of mutant males are always internal (MGI Ref ID J:67910)
craniofacial phenotype
- abnormal jaw morphology
- frequently exhibit misalignment of the jaws (MGI Ref ID J:67910)
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
endocrine/exocrine gland phenotype
- cryptorchism
- testes of mutant males are always internal (MGI Ref ID J:67910)
skeleton phenotype
- abnormal jaw morphology
- frequently exhibit misalignment of the jaws (MGI Ref ID J:67910)
- abnormal tooth morphology
- frequently exhibit uneven wearing of the teeth (MGI Ref ID J:67910)
hearing/vestibular/ear phenotype
- abnormal hearing physiology
- some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected (MGI Ref ID J:67910)
Genotype: Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * C57BL/6J * FVB/N
behavior/neurological phenotype
- seizures
- electrographic seizures are measured in cultured neurons from constitutive null mice (MGI Ref ID J:166851)
nervous system phenotype
- abnormal miniature inhibitory postsynaptic currents
- electrographic seizures are measured in cultured neurons from constitutive null mice (MGI Ref ID J:166851)
- abnormal nervous system electrophysiology
- non-seizure hyperexcitability discharges are observed in recordings from in cultured neurons from constitutive null mice (MGI Ref ID J:166851)
- seizures
- electrographic seizures are measured in cultured neurons from constitutive null mice (MGI Ref ID J:166851)
The following phenotype relates to a compound genotype created using this strain
Genotype: Mecp2tm1.1Bird/Y Tg(MECP2)1Hzo/0
involves: 129P2/OlaHsd * FVB
mortality/aging
- mortality/aging
- premature death in male Mecp2-null mice at 10-11 weeks is rescued (MGI Ref ID J:94407)
behavior/neurological phenotype
- behavior/neurological phenotype
- neurological abnormalities seen in single transgenic mutant are rescued by loss of Mecp2 (MGI Ref ID J:94407)
nervous system phenotype
- nervous system phenotype
- amplitudes of EPSCs, RRP size, and mEPSC frequency, amplitude, and decay kinetics are normalized in double mutant brains compared to either single mutant brain (MGI Ref ID J:126964)
- glutamatergic synaptic density is normalized in double mutant brains compared to either single mutant brain (MGI Ref ID J:126964)
References
- 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: 11242117MGI: J:67910
- Samaco RC; McGraw CM; Ward CS; Sun Y; Neul JL; Zoghbi HY. 2013. Female Mecp2+/- mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies. Hum Mol Genet 22(1):96-109. PubMed: 23026749MGI: J:189576
Additional References
- Braunschweig D; Simcox T; Samaco RC; LaSalle JM. 2004. X-Chromosome inactivation ratios affect wild-type MeCP2 expression within mosaic Rett syndrome and Mecp2-/+ mouse brain. Hum Mol Genet 13(12):1275-86. PubMed: 15115765MGI: J:91193
Additional - Mecp2tm1.1Bird related
- Abdala AP; Dutschmann M; Bissonnette JM; Paton JF. 2010. Correction of respiratory disorders in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A 107(42):18208-13. PubMed: 20921395MGI: J:165539
- Abdala AP; Lioy DT; Garg SK; Knopp SJ; Paton JF; Bissonnette JM. 2014. Effect of Sarizotan, a 5-HT1a and D2-like receptor agonist, on respiration in three mouse models of Rett syndrome. Am J Respir Cell Mol Biol 50(6):1031-9. PubMed: 24351104MGI: J:232165
- Alvarez-Saavedra M; Saez MA; Kang D; Zoghbi HY; Young JI. 2007. Cell-specific expression of wild-type MeCP2 in mouse models of Rett syndrome yields insight about pathogenesis. Hum Mol Genet 16(19):2315-25. PubMed: 17635839MGI: J:124365
- Asaka Y; Jugloff DG; Zhang L; Eubanks JH; Fitzsimonds RM. 2006. Hippocampal synaptic plasticity is impaired in the Mecp2-null mouse model of Rett syndrome. Neurobiol Dis 21(1):217-27. PubMed: 16087343MGI: J:104545
- Bahey NG; Gadalla KKE; McGonigal R; Bailey MES; Edgar JM; Cobb SR. 2017. Reduced axonal diameter of peripheral nerve fibers in a mouse model of Rett syndrome. Neuroscience 358(None):261-268. PubMed: 28687309MGI: J:249751
- Baker SA; Chen L; Wilkins AD; Yu P; Lichtarge O; Zoghbi HY. 2013. An AT-Hook Domain in MeCP2 Determines the Clinical Course of Rett Syndrome and Related Disorders. Cell 152(5):984-96. PubMed: 23452848MGI: J:194039
- Banerjee A; Rikhye RV; Breton-Provencher V; Tang X; Li C; Li K; Runyan CA; Fu Z; Jaenisch R; Sur M. 2016. Jointly reduced inhibition and excitation underlies circuit-wide changes in cortical processing in Rett syndrome. Proc Natl Acad Sci U S A 113(46):E7287-E7296. PubMed: 27803317MGI: J:238847
- Bedogni F; Cobolli Gigli C; Pozzi D; Rossi RL; Scaramuzza L; Rossetti G; Pagani M; Kilstrup-Nielsen C; Matteoli M; Landsberger N. 2016. Defects During Mecp2 Null Embryonic Cortex Development Precede the Onset of Overt Neurological Symptoms. Cereb Cortex 26(6):2517-2529. PubMed: 25979088MGI: J:242531
- Belichenko NP; Belichenko PV; Mobley WC. 2009. Evidence for both neuronal cell autonomous and nonautonomous effects of methyl-CpG-binding protein 2 in the cerebral cortex of female mice with Mecp2 mutation. Neurobiol Dis 34(1):71-7. PubMed: 19167498MGI: J:147302
- Berghoff EG; Clark MF; Chen S; Cajigas I; Leib DE; Kohtz JD. 2013. Evf2 (Dlx6as) lncRNA regulates ultraconserved enhancer methylation and the differential transcriptional control of adjacent genes. Development 140(21):4407-16. PubMed: 24089468MGI: J:204597
- Bissonnette JM; Knopp SJ. 2008. Effect of inspired oxygen on periodic breathing in methy-CpG-binding protein 2 (Mecp2) deficient mice. J Appl Physiol 104(1):198-204. PubMed: 18006868MGI: J:149046
- Bissonnette JM; Knopp SJ; Maylie J; Thong T. 2007. Autonomic cardiovascular control in methyl-CpG-binding protein 2 (Mecp2) deficient mice. Auton Neurosci 136(1-2):82-9. PubMed: 17544925MGI: J:129856
- Blue ME; Boskey AL; Doty SB; Fedarko NS; Hossain MA; Shapiro JR. 2015. Osteoblast function and bone histomorphometry in a murine model of Rett syndrome. Bone 76:23-30. PubMed: 25769649MGI: J:223784
- Blue ME; Kaufmann WE; Bressler J; Eyring C; O'driscoll C; Naidu S; Johnston MV. 2011. Temporal and Regional Alterations in NMDA Receptor Expression in Mecp2-Null Mice. Anat Rec (Hoboken) 294(10):1624-34. PubMed: 21901842MGI: J:176669
- Braun S; Kottwitz D; Nuber UA. 2012. Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome. Hum Mol Genet 21(8):1673-80. PubMed: 22186023MGI: J:181897
- Braunschweig D; Simcox T; Samaco RC; LaSalle JM. 2004. X-Chromosome inactivation ratios affect wild-type MeCP2 expression within mosaic Rett syndrome and Mecp2-/+ mouse brain. Hum Mol Genet 13(12):1275-86. PubMed: 15115765MGI: J:91193
- Buchovecky CM; Turley SD; Brown HM; Kyle SM; McDonald JG; Liu B; Pieper AA; Huang W; Katz DM; Russell DW; Shendure J; Justice MJ. 2013. A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett Syndrome Nat Genet 45(9):1013-20. PubMed: 23892605MGI: J:198551
- Caballero IM; Hendrich B. 2005. MeCP2 in neurons: closing in on the causes of Rett syndrome. Hum Mol Genet 14 Spec No 1:R19-26. PubMed: 15809268MGI: J:97524
- Castro J; Garcia RI; Kwok S; Banerjee A; Petravicz J; Woodson J; Mellios N; Tropea D; Sur M. 2014. Functional recovery with recombinant human IGF1 treatment in a mouse model of Rett Syndrome. Proc Natl Acad Sci U S A 111(27):9941-6. PubMed: 24958891MGI: J:212165
- Chahrour M; Jung SY; Shaw C; Zhou X; Wong ST; Qin J; Zoghbi HY. 2008. MeCP2, a key contributor to neurological disease, activates and represses transcription. Science 320(5880):1224-9. PubMed: 18511691MGI: J:136439
- Chao HT; Chen H; Samaco RC; Xue M; Chahrour M; Yoo J; Neul JL; Gong S; Lu HC; Heintz N; Ekker M; Rubenstein JL; Noebels JL; Rosenmund C; Zoghbi HY. 2010. Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes. Nature 468(7321):263-9. PubMed: 21068835MGI: J:166851
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