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+29
(04-OCT-13) |
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 |
| $230.10 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
Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
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
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)
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
- 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
- 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
- Chao HT; Zoghbi HY; Rosenmund C. 2007. MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron 56(1):58-65. PubMed: 17920015MGI: J:126964
- Collins AL; Levenson JM; Vilaythong AP; Richman R; Armstrong DL; Noebels JL; David Sweatt J; Zoghbi HY. 2004. Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet 13(21):2679-89. PubMed: 15351775MGI: J:94407
- Conti V; Gandaglia A; Galli F; Tirone M; Bellini E; Campana L; Kilstrup-Nielsen C; Rovere-Querini P; Brunelli S; Landsberger N. 2015. MeCP2 Affects Skeletal Muscle Growth and Morphology through Non Cell-Autonomous Mechanisms. PLoS One 10(6):e0130183. PubMed: 26098633MGI: J:237891
- Cortelazzo A; De Felice C; Guerranti R; Signorini C; Leoncini S; Pecorelli A; Scalabri F; Madonna M; Filosa S; Della Giovampaola C; Capone A; Durand T; Mirasole C; Zolla L; Valacchi G; Ciccoli L; Guy J; D'Esposito M; Hayek J. 2016. Abnormal N-glycosylation pattern for brain nucleotide pyrophosphatase-5 (NPP-5) in Mecp2-mutant murine models of Rett syndrome. Neurosci Res 105:28-34. PubMed: 26476268MGI: J:231839
- Cronk JC; Derecki NC; Ji E; Xu Y; Lampano AE; Smirnov I; Baker W; Norris GT; Marin I; Coddington N; Wolf Y; Turner SD; Aderem A; Klibanov AL; Harris TH; Jung S; Litvak V; Kipnis J. 2015. Methyl-CpG Binding Protein 2 Regulates Microglia and Macrophage Gene Expression in Response to Inflammatory Stimuli. Immunity 42(4):679-91. PubMed: 25902482MGI: J:229774
- D'Cruz JA; Wu C; Zahid T; El-Hayek Y; Zhang L; Eubanks JH. 2010. Alterations of cortical and hippocampal EEG activity in MeCP2-deficient mice. Neurobiol Dis 38(1):8-16. PubMed: 20045053MGI: J:159940
- De Felice C; Della Ragione F; Signorini C; Leoncini S; Pecorelli A; Ciccoli L; Scalabri F; Marracino F; Madonna M; Belmonte G; Ricceri L; De Filippis B; Laviola G; Valacchi G; Durand T; Galano JM; Oger C; Guy A; Bultel-Ponce V; Guy J; Filosa S; Hayek J; D'Esposito M. 2014. Oxidative brain damage in Mecp2-mutant murine models of Rett syndrome. Neurobiol Dis 68:66-77. PubMed: 24769161MGI: J:214008
- Deng V; Matagne V; Banine F; Frerking M; Ohliger P; Budden S; Pevsner J; Dissen GA; Sherman LS; Ojeda SR. 2007. FXYD1 is an MeCP2 target gene overexpressed in the brains of Rett syndrome patients and Mecp2-null mice. Hum Mol Genet 16(6):640-50. PubMed: 17309881MGI: J:121717
- Deogracias R; Yazdani M; Dekkers MP; Guy J; Ionescu MC; Vogt KE; Barde YA. 2012. Fingolimod, a sphingosine-1 phosphate receptor modulator, increases BDNF levels and improves symptoms of a mouse model of Rett syndrome. Proc Natl Acad Sci U S A 109(35):14230-5. PubMed: 22891354MGI: J:188569
- Derecki NC; Cronk JC; Lu Z; Xu E; Abbott SB; Guyenet PG; Kipnis J. 2012. Wild-type microglia arrest pathology in a mouse model of Rett syndrome. Nature 484(7392):105-9. PubMed: 22425995MGI: J:183878
- Durand S; Patrizi A; Quast KB; Hachigian L; Pavlyuk R; Saxena A; Carninci P; Hensch TK; Fagiolini M. 2012. NMDA receptor regulation prevents regression of visual cortical function in the absence of Mecp2. Neuron 76(6):1078-90. PubMed: 23259945MGI: J:197657
- Ebert DH; Gabel HW; Robinson ND; Kastan NR; Hu LS; Cohen S; Navarro AJ; Lyst MJ; Ekiert R; Bird AP; Greenberg ME. 2013. Activity-dependent phosphorylation of MeCP2 threonine 308 regulates interaction with NCoR. Nature 499(7458):341-5. PubMed: 23770587MGI: J:204723
- El-Khoury R; Panayotis N; Matagne V; Ghata A; Villard L; Roux JC. 2014. GABA and glutamate pathways are spatially and developmentally affected in the brain of Mecp2-deficient mice. PLoS One 9(3):e92169. PubMed: 24667344MGI: J:214151
- Fukuda T; Itoh M; Ichikawa T; Washiyama K; Goto Y. 2005. Delayed maturation of neuronal architecture and synaptogenesis in cerebral cortex of Mecp2-deficient mice. J Neuropathol Exp Neurol 64(6):537-44. PubMed: 15977646MGI: J:104939
- Gantz SC; Ford CP; Neve KA; Williams JT. 2011. Loss of mecp2 in substantia nigra dopamine neurons compromises the nigrostriatal pathway. J Neurosci 31(35):12629-37. PubMed: 21880923MGI: J:176217
- Gogolla N; Takesian AE; Feng G; Fagiolini M; Hensch TK. 2014. Sensory integration in mouse insular cortex reflects GABA circuit maturation. Neuron 83(4):894-905. PubMed: 25088363MGI: J:221545
- Gonzales ML; Adams S; Dunaway KW; LaSalle JM. 2012. Phosphorylation of distinct sites in MeCP2 modifies cofactor associations and the dynamics of transcriptional regulation. Mol Cell Biol 32(14):2894-903. PubMed: 22615490MGI: J:186652
- Hao S; Tang B; Wu Z; Ure K; Sun Y; Tao H; Gao Y; Patel AJ; Curry DJ; Samaco RC; Zoghbi HY; Tang J. 2015. Forniceal deep brain stimulation rescues hippocampal memory in Rett syndrome mice. Nature 526(7573):430-4. PubMed: 26469053MGI: J:226766
- Heckman LD; Chahrour MH; Zoghbi HY. 2014. Rett-causing mutations reveal two domains critical for MeCP2 function and for toxicity in MECP2 duplication syndrome mice. Elife 3:. PubMed: 24970834MGI: J:214143
- Herrera JA; Ward CS; Pitcher MR; Percy AK; Skinner S; Kaufmann WE; Glaze DG; Wehrens XH; Neul JL. 2015. Treatment of cardiac arrhythmias in a mouse model of Rett syndrome with Na+-channel-blocking antiepileptic drugs. Dis Model Mech 8(4):363-71. PubMed: 25713300MGI: J:221198
- Ho EC; Eubanks JH; Zhang L; Skinner FK. 2014. Network models predict that reduced excitatory fluctuations can give rise to hippocampal network hyper-excitability in MeCP2-null mice. PLoS One 9(3):e91148. PubMed: 24642514MGI: J:215080
- Horike S; Cai S; Miyano M; Cheng JF; Kohwi-Shigematsu T. 2005. Loss of silent-chromatin looping and impaired imprinting of DLX5 in Rett syndrome. Nat Genet 37(1):31-40. PubMed: 15608638MGI: J:96437
- Hulbert SW; Jiang YH. 2016. Monogenic mouse models of autism spectrum disorders: Common mechanisms and missing links. Neuroscience 321:3-23. PubMed: 26733386MGI: J:232349
- Ide S; Itoh M; Goto Y. 2005. Defect in normal developmental increase of the brain biogenic amine concentrations in the mecp2-null mouse. Neurosci Lett 386(1):14-7. PubMed: 15975715MGI: J:102566
- Isoda K; Morimoto M; Matsui F; Hasegawa T; Tozawa T; Morioka S; Chiyonobu T; Nishimura A; Yoshimoto K; Hosoi H. 2010. Postnatal changes in serotonergic innervation to the hippocampus of methyl-CpG-binding protein 2-null mice. Neuroscience 165(4):1254-60. PubMed: 19932741MGI: J:159547
- Itoh M; Tahimic CG; Ide S; Otsuki A; Sasaoka T; Noguchi S; Oshimura M; Goto Y; Kurimasa A. 2012. Methyl CpG-binding protein isoform MeCP2_e2 is dispensable for Rett syndrome phenotypes but essential for embryo viability and placenta development. J Biol Chem 287(17):13859-67. PubMed: 22375006MGI: J:184364
- Jin LW; Horiuchi M; Wulff H; Liu XB; Cortopassi GA; Erickson JD; Maezawa I. 2015. Dysregulation of glutamine transporter SNAT1 in Rett syndrome microglia: a mechanism for mitochondrial dysfunction and neurotoxicity. J Neurosci 35(6):2516-29. PubMed: 25673846MGI: J:219878
- Jin X; Cui N; Zhong W; Jin XT; Jiang C. 2013. GABAergic synaptic inputs of locus coeruleus neurons in wild-type and Mecp2-null mice. Am J Physiol Cell Physiol 304(9):C844-57. PubMed: 23392116MGI: J:198005
- Jin X; Zhong W; Jiang C. 2013. Time-dependent modulation of GABA(A)-ergic synaptic transmission by allopregnanolone in locus coeruleus neurons of Mecp2-null mice. Am J Physiol Cell Physiol 305(11):C1151-60. PubMed: 24067915MGI: J:210196
- Johnson CM; Zhong W; Cui N; Wu Y; Xing H; Zhang S; Jiang C. 2016. Defects in brainstem neurons associated with breathing and motor function in the Mecp2R168X/Y mouse model of Rett syndrome. Am J Physiol Cell Physiol 311(6):C895-C909. PubMed: 27653984MGI: J:239338
- Jordan C; Francke U. 2006. Ube3a expression is not altered in Mecp2 mutant mice. Hum Mol Genet 15(14):2210-5. PubMed: 16754645MGI: J:112026
- Jugloff DG; Logan R; Eubanks JH. 2006. Breeding and maintenance of an Mecp2-deficient mouse model of Rett Syndrome J Neurosci Methods 154(1-2):89-95. PubMed: 16439027MGI: J:106418
- Jugloff DG; Vandamme K; Logan R; Visanji NP; Brotchie JM; Eubanks JH. 2008. Targeted delivery of an Mecp2 transgene to forebrain neurons improves the behavior of female Mecp2-deficient mice. Hum Mol Genet 17(10):1386-96. PubMed: 18223199MGI: J:135351
- Kerr B; Alvarez-Saavedra M; Saez MA; Saona A; Young JI. 2008. Defective body-weight regulation, motor control and abnormal social interactions in Mecp2 hypomorphic mice. Hum Mol Genet 17(12):1707-17. PubMed: 18321865MGI: J:135824
- Kerr B; Silva PA; Walz K; Young JI. 2010. Unconventional transcriptional response to environmental enrichment in a mouse model of Rett syndrome. PLoS One 5(7):e11534. PubMed: 20634955MGI: J:163111
- Kerr B; Soto C J; Saez M; Abrams A; Walz K; Young JI. 2012. Transgenic complementation of MeCP2 deficiency: phenotypic rescue of Mecp2-null mice by isoform-specific transgenes. Eur J Hum Genet 20(1):69-76. PubMed: 21829232MGI: J:234600
- Kishi N; MacDonald JL; Ye J; Molyneaux BJ; Azim E; Macklis JD. 2016. Reduction of aberrant NF-kappaB signalling ameliorates Rett syndrome phenotypes in Mecp2-null mice. Nat Commun 7:10520. PubMed: 26821816MGI: J:236294
- Kriaucionis S; Paterson A; Curtis J; Guy J; Macleod N; Bird A. 2006. Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndrome. Mol Cell Biol 26(13):5033-42. PubMed: 16782889MGI: J:110323
- Krishnan K; Wang BS; Lu J; Wang L; Maffei A; Cang J; Huang ZJ. 2015. MeCP2 regulates the timing of critical period plasticity that shapes functional connectivity in primary visual cortex. Proc Natl Acad Sci U S A 112(34):E4782-91. PubMed: 26261347MGI: J:226639
- Krishnan N; Krishnan K; Connors CR; Choy MS; Page R; Peti W; Van Aelst L; Shea SD; Tonks NK. 2015. PTP1B inhibition suggests a therapeutic strategy for Rett syndrome. J Clin Invest 125(8):3163-77. PubMed: 26214522MGI: J:225802
- Kron M; Muller M. 2010. Impaired hippocampal Ca2+ homeostasis and concomitant K+ channel dysfunction in a mouse model of Rett syndrome during anoxia. Neuroscience 171(1):300-15. PubMed: 20732392MGI: J:169732
- Kyle SM; Saha PK; Brown HM; Chan LC; Justice MJ. 2016. MeCP2 co-ordinates liver lipid metabolism with the NCoR1/HDAC3 corepressor complex. Hum Mol Genet 25(14):3029-3041. PubMed: 27288453MGI: J:237141
- Lee W; Yun JM; Woods R; Dunaway K; Yasui DH; Lasalle JM; Gong Q. 2014. MeCP2 regulates activity-dependent transcriptional responses in olfactory sensory neurons. Hum Mol Genet 23(23):6366-74. PubMed: 25008110MGI: J:215637
- Liao W; Gandal MJ; Ehrlichman RS; Siegel SJ; Carlson GC. 2012. MeCP2+/- mouse model of RTT reproduces auditory phenotypes associated with Rett syndrome and replicate select EEG endophenotypes of autism spectrum disorder. Neurobiol Dis 46(1):88-92. PubMed: 22249109MGI: J:182312
- Linhoff MW; Garg SK; Mandel G. 2015. A High-Resolution Imaging Approach to Investigate Chromatin Architecture in Complex Tissues. Cell 163(1):246-55. PubMed: 26406379MGI: J:224900
- Lioy DT; Garg SK; Monaghan CE; Raber J; Foust KD; Kaspar BK; Hirrlinger PG; Kirchhoff F; Bissonnette JM; Ballas N; Mandel G. 2011. A role for glia in the progression of Rett's syndrome. Nature 475(7357):497-500. PubMed: 21716289MGI: J:174777
- Lombardi LM; Baker SA; Zoghbi HY. 2015. MECP2 disorders: from the clinic to mice and back. J Clin Invest 125(8):2914-23. PubMed: 26237041MGI: J:225796
- Macdonald JL; Verster A; Berndt A; Roskams AJ. 2010. MBD2 and MeCP2 regulate distinct transitions in the stage-specific differentiation of olfactory receptor neurons. Mol Cell Neurosci 44(1):55-67. PubMed: 20188178MGI: J:164144
- Maezawa I; Jin LW. 2010. Rett syndrome microglia damage dendrites and synapses by the elevated release of glutamate. J Neurosci 30(15):5346-56. PubMed: 20392956MGI: J:159848
- Maezawa I; Swanberg S; Harvey D; LaSalle JM; Jin LW. 2009. Rett syndrome astrocytes are abnormal and spread MeCP2 deficiency through gap junctions. J Neurosci 29(16):5051-61. PubMed: 19386901MGI: J:158156
- Makedonski K; Abuhatzira L; Kaufman Y; Razin A; Shemer R. 2005. MeCP2 deficiency in Rett syndrome causes epigenetic aberrations at the PWS/AS imprinting center that affects UBE3A expression. Hum Mol Genet 14(8):1049-58. PubMed: 15757975MGI: J:98000
- Martin Caballero I; Hansen J; Leaford D; Pollard S; Hendrich BD. 2009. The methyl-CpG binding proteins Mecp2, Mbd2 and Kaiso are dispensable for mouse embryogenesis, but play a redundant function in neural differentiation. PLoS One 4(1):e4315. PubMed: 19177165MGI: J:144819
- McCauley MD; Wang T; Mike E; Herrera J; Beavers DL; Huang TW; Ward CS; Skinner S; Percy AK; Glaze DG; Wehrens XH; Neul JL. 2011. Pathogenesis of lethal cardiac arrhythmias in Mecp2 mutant mice: implication for therapy in Rett syndrome. Sci Transl Med 3(113):113ra125. PubMed: 22174313MGI: J:183640
- Medrihan L; Tantalaki E; Aramuni G; Sargsyan V; Dudanova I; Missler M; Zhang W. 2008. Early defects of GABAergic synapses in the brain stem of a MeCP2 mouse model of Rett syndrome. J Neurophysiol 99(1):112-21. PubMed: 18032561MGI: J:147675
- Mellen M; Ayata P; Dewell S; Kriaucionis S; Heintz N. 2012. MeCP2 binds to 5hmC enriched within active genes and accessible chromatin in the nervous system. Cell 151(7):1417-30. PubMed: 23260135MGI: J:193327
- Mellios N; Woodson J; Garcia RI; Crawford B; Sharma J; Sheridan SD; Haggarty SJ; Sur M. 2014. beta2-Adrenergic receptor agonist ameliorates phenotypes and corrects microRNA-mediated IGF1 deficits in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A 111(27):9947-52. PubMed: 24958851MGI: J:212180
- Metcalf BM; Mullaney BC; Johnston MV; Blue ME. 2006. Temporal shift in methyl-CpG binding protein 2 expression in a mouse model of Rett syndrome. Neuroscience 139(4):1449-60. PubMed: 16549272MGI: J:108953
- Miralves J; Magdeleine E; Kaddoum L; Brun H; Peries S; Joly E. 2007. High Levels of MeCP2 Depress MHC Class I Expression in Neuronal Cells. PLoS One 2(12):e1354. PubMed: 18159237MGI: J:130960
- Mironov SL; Skorova E; Hartelt N; Mironova LA; Hasan MT; Kugler S. 2009. Remodelling of the respiratory network in a mouse model of Rett syndrome depends on brain-derived neurotrophic factor regulated slow calcium buffering. J Physiol 587(Pt 11):2473-85. PubMed: 19359374MGI: J:176540
- Nan X; Hou J; Maclean A; Nasir J; Lafuente MJ; Shu X; Kriaucionis S; Bird A. 2007. Interaction between chromatin proteins MECP2 and ATRX is disrupted by mutations that cause inherited mental retardation. Proc Natl Acad Sci U S A 104(8):2709-14. PubMed: 17296936MGI: J:125897
- Nelson ED; Kavalali ET; Monteggia LM. 2006. MeCP2-dependent transcriptional repression regulates excitatory neurotransmission. Curr Biol 16(7):710-6. PubMed: 16581518MGI: J:107739
- Nott A; Cheng J; Gao F; Lin YT; Gjoneska E; Ko T; Minhas P; Zamudio AV; Meng J; Zhang F; Jin P; Tsai LH. 2016. Histone deacetylase 3 associates with MeCP2 to regulate FOXO and social behavior. Nat Neurosci 19(11):1497-1505. PubMed: 27428650MGI: J:238124
- Noutel J; Hong YK; Leu B; Kang E; Chen C. 2011. Experience-dependent retinogeniculate synapse remodeling is abnormal in MeCP2-deficient mice. Neuron 70(1):35-42. PubMed: 21482354MGI: J:174711
- O'Connor RD; Zayzafoon M; Farach-Carson MC; Schanen NC. 2009. Mecp2 deficiency decreases bone formation and reduces bone volume in a rodent model of Rett syndrome. Bone 45(2):346-56. PubMed: 19414073MGI: J:226599
- Oginsky MF; Cui N; Zhong W; Johnson CM; Jiang C. 2014. Alterations in the cholinergic system of brain stem neurons in a mouse model of Rett syndrome. Am J Physiol Cell Physiol 307(6):C508-20. PubMed: 25009110MGI: J:221827
- Orefice LL; Zimmerman AL; Chirila AM; Sleboda SJ; Head JP; Ginty DD. 2016. Peripheral Mechanosensory Neuron Dysfunction Underlies Tactile and Behavioral Deficits in Mouse Models of ASDs. Cell :. PubMed: 27293187MGI: J:232023
- Panayotis N; Pratte M; Borges-Correia A; Ghata A; Villard L; Roux JC. 2011. Morphological and functional alterations in the substantia nigra pars compacta of the Mecp2-null mouse. Neurobiol Dis 41(2):385-97. PubMed: 20951208MGI: J:168648
- Peddada S; Yasui DH; LaSalle JM. 2006. Inhibitors of differentiation (ID1, ID2, ID3 and ID4) genes are neuronal targets of MeCP2 that are elevated in Rett syndrome. Hum Mol Genet 15(12):2003-14. PubMed: 16682435MGI: J:112064
- Pitcher MR; Ward CS; Arvide EM; Chapleau CA; Pozzo-Miller L; Hoeflich A; Sivaramakrishnan M; Saenger S; Metzger F; Neul JL. 2013. Insulinotropic treatments exacerbate metabolic syndrome in mice lacking MeCP2 function. Hum Mol Genet 22(13):2626-33. PubMed: 23462290MGI: J:198235
- Pratte M; Panayotis N; Ghata A; Villard L; Roux JC. 2011. Progressive motor and respiratory metabolism deficits in post-weaning Mecp2-null male mice. Behav Brain Res 216(1):313-20. PubMed: 20713094MGI: J:165732
- Rastegar M; Hotta A; Pasceri P; Makarem M; Cheung AY; Elliott S; Park KJ; Adachi M; Jones FS; Clarke ID; Dirks P; Ellis J. 2009. MECP2 isoform-specific vectors with regulated expression for Rett syndrome gene therapy. PLoS One 4(8):e6810. PubMed: 19710912MGI: J:152398
- Roux JC; Dura E; Villard L. 2008. Tyrosine hydroxylase deficit in the chemoafferent and the sympathoadrenergic pathways of the Mecp2 deficient mouse. Neurosci Lett 447(1):82-6. PubMed: 18834926MGI: J:143308
- Roux JC; Zala D; Panayotis N; Borges-Correia A; Saudou F; Villard L. 2012. Modification of Mecp2 dosage alters axonal transport through the Huntingtin/Hap1 pathway. Neurobiol Dis 45(2):786-95. PubMed: 22127389MGI: J:182049
- Rube HT; Lee W; Hejna M; Chen H; Yasui DH; Hess JF; LaSalle JM; Song JS; Gong Q. 2016. Sequence features accurately predict genome-wide MeCP2 binding in vivo. Nat Commun 7:11025. PubMed: 27008915MGI: J:236741
- Rusconi F; Paganini L; Braida D; Ponzoni L; Toffolo E; Maroli A; Landsberger N; Bedogni F; Turco E; Pattini L; Altruda F; De Biasi S; Sala M; Battaglioli E. 2015. LSD1 Neurospecific Alternative Splicing Controls Neuronal Excitability in Mouse Models of Epilepsy. Cereb Cortex 25(9):2729-40. PubMed: 24735673MGI: J:235553
- Russell JC; Blue ME; Johnston MV; Naidu S; Hossain MA. 2007. Enhanced cell death in MeCP2 null cerebellar granule neurons exposed to excitotoxicity and hypoxia. Neuroscience 150(3):563-74. PubMed: 17997046MGI: J:130772
- Samaco RC; Mandel-Brehm C; Chao HT; Ward CS; Fyffe-Maricich SL; Ren J; Hyland K; Thaller C; Maricich SM; Humphreys P; Greer JJ; Percy A; Glaze DG; Zoghbi HY; Neul JL. 2009. Loss of MeCP2 in aminergic neurons causes cell-autonomous defects in neurotransmitter synthesis and specific behavioral abnormalities. Proc Natl Acad Sci U S A :. PubMed: 20007372MGI: J:155808
- Samaco RC; Mandel-Brehm C; McGraw CM; Shaw CA; McGill BE; Zoghbi HY. 2012. Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome. Nat Genet 44(2):206-11. PubMed: 22231481MGI: J:181213
- Santos M; Silva-Fernandes A; Oliveira P; Sousa N; Maciel P. 2007. Evidence for abnormal early development in a mouse model of Rett syndrome. Genes Brain Behav 6(3):277-86. PubMed: 16848781MGI: J:135089
- Santos M; Summavielle T; Teixeira-Castro A; Silva-Fernandes A; Duarte-Silva S; Marques F; Martins L; Dierssen M; Oliveira P; Sousa N; Maciel P. 2010. Monoamine deficits in the brain of methyl-CpG binding protein 2 null mice suggest the involvement of the cerebral cortex in early stages of Rett syndrome. Neuroscience 170(2):453-67. PubMed: 20633611MGI: J:165306
- Saywell V; Viola A; Confort-Gouny S; Le Fur Y; Villard L; Cozzone PJ. 2006. Brain magnetic resonance study of Mecp2 deletion effects on anatomy and metabolism. Biochem Biophys Res Commun 340(3):776-83. PubMed: 16380085MGI: J:104731
- Schafer DP; Heller CT; Gunner G; Heller M; Gordon C; Hammond T; Wolf Y; Jung S; Stevens B. 2016. Microglia contribute to circuit defects in Mecp2 null mice independent of microglia-specific loss of Mecp2 expression. Elife 5:. PubMed: 27458802MGI: J:235289
- Schule B; Li HH; Fisch-Kohl C; Purmann C; Francke U. 2007. DLX5 and DLX6 expression is biallelic and not modulated by MeCP2 deficiency. Am J Hum Genet 81(3):492-506. PubMed: 17701895MGI: J:134837
- Schwartzer JJ; Koenig CM; Berman RF. 2013. Using mouse models of autism spectrum disorders to study the neurotoxicology of gene-environment interactions. Neurotoxicol Teratol 36:17-35. PubMed: 23010509MGI: J:221700
- Singleton MK; Gonzales ML; Leung KN; Yasui DH; Schroeder DI; Dunaway K; LaSalle JM. 2011. MeCP2 is required for global heterochromatic and nucleolar changes during activity-dependent neuronal maturation. Neurobiol Dis 43(1):190-200. PubMed: 21420494MGI: J:174326
- Stettner GM; Huppke P; Brendel C; Richter DW; Gartner J; Dutschmann M. 2007. Breathing dysfunctions associated with impaired control of postinspiratory activity in Mecp2-/y knockout mice. J Physiol 579(Pt 3):863-76. PubMed: 17204503MGI: J:140841
- Szulwach KE; Li X; Li Y; Song CX; Wu H; Dai Q; Irier H; Upadhyay AK; Gearing M; Levey AI; Vasanthakumar A; Godley LA; Chang Q; Cheng X; He C; Jin P. 2011. 5-hmC-mediated epigenetic dynamics during postnatal neurodevelopment and aging. Nat Neurosci 14(12):1607-16. PubMed: 22037496MGI: J:180327
- Tao J; Wu H; Coronado AA; de Laittre E; Osterweil EK; Zhang Y; Bear MF. 2016. Negative Allosteric Modulation of mGluR5 Partially Corrects Pathophysiology in a Mouse Model of Rett Syndrome. J Neurosci 36(47):11946-11958. PubMed: 27881780MGI: J:237191
- Toloe J; Mollajew R; Kugler S; Mironov SL. 2014. Metabolic differences in hippocampal 'Rett' neurons revealed by ATP imaging. Mol Cell Neurosci 59:47-56. PubMed: 24394521MGI: J:215711
- Vermehren-Schmaedick A; Jenkins VK; Knopp SJ; Balkowiec A; Bissonnette JM. 2012. Acute intermittent hypoxia-induced expression of brain-derived neurotrophic factor is disrupted in the brainstem of methyl-CpG-binding protein 2 null mice. Neuroscience 206:1-6. PubMed: 22297041MGI: J:184632
- Viemari JC; Roux JC; Tryba AK; Saywell V; Burnet H; Pena F; Zanella S; Bevengut M; Barthelemy-Requin M; Herzing LB; Moncla A; Mancini J; Ramirez JM; Villard L; Hilaire G. 2005. Mecp2 deficiency disrupts norepinephrine and respiratory systems in mice. J Neurosci 25(50):11521-30. PubMed: 16354910MGI: J:104053
- Villani C; Sacchetti G; Bagnati R; Passoni A; Fusco F; Carli M; Invernizzi RW. 2016. Lovastatin fails to improve motor performance and survival in methyl-CpG-binding protein2-null mice. Elife 5:. PubMed: 27892851MGI: J:239171
- Viola A; Saywell V; Villard L; Cozzone PJ; Lutz NW. 2007. Metabolic fingerprints of altered brain growth, osmoregulation and neurotransmission in a Rett syndrome model. PLoS One 2(1):e157. PubMed: 17237885MGI: J:129328
- Voituron N; Zanella S; Menuet C; Dutschmann M; Hilaire G. 2009. Early breathing defects after moderate hypoxia or hypercapnia in a mouse model of Rett syndrome. Respir Physiol Neurobiol 168(1-2):109-18. PubMed: 19524074MGI: J:155642
- Wang IT; Reyes AR; Zhou Z. 2013. Neuronal morphology in MeCP2 mouse models is intrinsically variable and depends on age, cell type, and Mecp2 mutation. Neurobiol Dis 58C:3-12. PubMed: 23659895MGI: J:197982
- Ward CS; Arvide EM; Huang TW; Yoo J; Noebels JL; Neul JL. 2011. MeCP2 Is Critical within HoxB1-Derived Tissues of Mice for Normal Lifespan. J Neurosci 31(28):10359-70. PubMed: 21753013MGI: J:174516
- Wither RG; Lang M; Zhang L; Eubanks JH. 2013. Regional MeCP2 expression levels in the female MeCP2-deficient mouse brain correlate with specific behavioral impairments. Exp Neurol 239:49-59. PubMed: 23022455MGI: J:196997
- Wood L; Shepherd GM. 2010. Synaptic circuit abnormalities of motor-frontal layer 2/3 pyramidal neurons in a mutant mouse model of Rett syndrome. Neurobiol Dis 38(2):281-7. PubMed: 20138994MGI: J:159917
- Yazdani M; Deogracias R; Guy J; Poot RA; Bird A; Barde YA. 2012. Disease modeling using embryonic stem cells: MeCP2 regulates nuclear size and RNA synthesis in neurons. Stem Cells 30(10):2128-39. PubMed: 22865604MGI: J:194651
- Zachariah RM; Olson CO; Ezeonwuka C; Rastegar M. 2012. Novel MeCP2 isoform-specific antibody reveals the endogenous MeCP2E1 expression in murine brain, primary neurons and astrocytes. PLoS One 7(11):e49763. PubMed: 23185431MGI: J:225070
- Zhang X; Cui N; Wu Z; Su J; Tadepalli JS; Sekizar S; Jiang C. 2010. Intrinsic membrane properties of locus coeruleus neurons in Mecp2-null mice. Am J Physiol Cell Physiol 298(3):C635-46. PubMed: 20042730MGI: J:157650
- Zhang X; Su J; Cui N; Gai H; Wu Z; Jiang C. 2011. The disruption of central CO2 chemosensitivity in a mouse model of Rett syndrome. Am J Physiol Cell Physiol 301(3):C729-38. PubMed: 21307341MGI: J:175673
- Zhang X; Su J; Rojas A; Jiang C. 2010. Pontine norepinephrine defects in Mecp2-null mice involve deficient expression of dopamine beta-hydroxylase but not a loss of catecholaminergic neurons. Biochem Biophys Res Commun 394(2):285-90. PubMed: 20193660MGI: J:158815
- Zhong W; Cui N; Jin X; Oginsky MF; Wu Y; Zhang S; Bondy B; Johnson CM; Jiang C. 2015. Methyl CpG Binding Protein 2 Gene Disruption Augments Tonic Currents of gamma-Aminobutyric Acid Receptors in Locus Coeruleus Neurons: IMPACT ON NEURONAL EXCITABILITY AND BREATHING. J Biol Chem 290(30):18400-11. PubMed: 25979331MGI: J:224611