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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Mecp2tm1.1Bird
targeted mutation 1.1, Adrian Bird
MGI:2137311
Summary 18 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Mecp2tm1.1Bird/Mecp2tm1.1Bird involves: 129P2/OlaHsd * C57BL/6 MGI:3624717
ht2
Mecp2tm1.1Bird/Mecp2+ involves: 129P2/OlaHsd MGI:3817236
ht3
Mecp2tm1.1Bird/Mecp2+ involves: 129P2/OlaHsd * C57BL/6 MGI:3624718
cx4
Mecp2tm1.1Bird/Y
Sum5M1Jus/Sum5+
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J MGI:5499848
cx5
Mecp2tm1.1Bird/Y
SqleSum3-Jus/Sqle+
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J MGI:5499839
cx6
Mecp2tm1.1Bird/Y
Sum1M1Jus/Sum1+
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J MGI:5499841
cx7
Mecp2tm1.1Bird/Y
Sum2M1Jus/Sum2+
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J MGI:5499846
cx8
Mecp2tm1.1Bird/Y
Sum4M1Jus/Sum4+
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J MGI:5499847
cx9
Mbd2tm1Bh/Mbd2tm1Bh
Mecp2tm1.1Bird/Y
involves: 129P2/OlaHsd * C57BL/6 MGI:3624722
cx10
Mecp2tm1.1Bird/Y
Tg(MECP2)1Hzo/0
involves: 129P2/OlaHsd * FVB MGI:3817185
cx11
Mecp2tm1.1Bird/Y
Tg(MECP2*R270X/GFP)#Hzo/0
involves: 129P2/OlaHsd * FVB MGI:5491040
cx12
Mecp2tm1.1Bird/Y
Tg(MECP2*G273X/GFP)#Hzo/0
involves: 129P2/OlaHsd * FVB MGI:5491041
ot13
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd MGI:3817230
ot14
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd * 129S6/SvEvTac MGI:5499849
ot15
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd * C57BL/6 MGI:3624713
ot16
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd * C57BL/6 * CBA MGI:3817273
ot17
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd * C57BL/6J * FVB/N MGI:4999648
ot18
Mecp2tm1.1Bird/Y involves: 129P2/OlaHsd * FVB MGI:5491051


Genotype
MGI:3624717
hm1
Allelic
Composition
Mecp2tm1.1Bird/Mecp2tm1.1Bird
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• variable progression of symptoms leads to rapid weight loss and death at about 54 days of age

behavior/neurological
• most mutants develop hindlimb clasping after 7 weeks of age
• exhibit reduced spontaneous movement between 3 and 8 weeks of age
• develop a stiff, uncoordinated gait between 3 and 8 weeks of age

growth/size/body
• frequently exhibit uneven wearing of the teeth
• variable progression of symptoms leads to rapid weight loss and death at about 54 days of age

respiratory system
• most mutants exhibit irregular breathing after 3-8 weeks of age

craniofacial
• frequently exhibit misalignment of the jaws
• frequently exhibit uneven wearing of the teeth

skeleton
• frequently exhibit misalignment of the jaws
• frequently exhibit uneven wearing of the teeth

hearing/vestibular/ear
• some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:67910




Genotype
MGI:3817236
ht2
Allelic
Composition
Mecp2tm1.1Bird/Mecp2+
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• decrease in the number of ATRX containing foci in null cells only
• decrease in the number of ATRX containing foci in null cells only
• 7-9 week old adults have 33% the number of Mecp2-expressing neurons compared to young adult wild-type
• 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

cellular
• X-chromosome inactivation becomes unbalance in older mutants, with a larger percentage expressing Mecp2 compared to younger mutants (>50%)




Genotype
MGI:3624718
ht3
Allelic
Composition
Mecp2tm1.1Bird/Mecp2+
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• acquire hindlimb clasping at ages greater than 3 months
• although heterozygous females initially show no symptoms and raise normal litters, they acquire inertia at ages greater than 3 months
• 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 controls

respiratory system
• often exhibit breathing irregularities by 9 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:67910




Genotype
MGI:5499848
cx4
Allelic
Composition
Mecp2tm1.1Bird/Y
Sum5M1Jus/Sum5+
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Sum5M1Jus mutation (0 available); any Sum5 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan in increased at least 3 weeks from the average 50% lethality time point of Mecp2tm1.1Bird hemizygotes

behavior/neurological
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

immune system
• unlike Mecp2tm1.1Bird hemizygotes

growth/size/body
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

vision/eye
• unlike Mecp2tm1.1Bird hemizygotes




Genotype
MGI:5499839
cx5
Allelic
Composition
Mecp2tm1.1Bird/Y
SqleSum3-Jus/Sqle+
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
SqleSum3-Jus mutation (0 available); any Sqle mutation (20 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan in increased at least 3 weeks from the average 50% lethality time point of Mecp2tm1.1Bird hemizygotes

behavior/neurological
• as in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

craniofacial
• as in Mecp2tm1.1Bird hemizygotes

growth/size/body
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

immune system
• unlike Mecp2tm1.1Bird hemizygotes

integument
• unlike Mecp2tm1.1Bird hemizygotes

respiratory system
• as in Mecp2tm1.1Bird hemizygotes

skeleton
• as in Mecp2tm1.1Bird hemizygotes




Genotype
MGI:5499841
cx6
Allelic
Composition
Mecp2tm1.1Bird/Y
Sum1M1Jus/Sum1+
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Sum1M1Jus mutation (0 available); any Sum1 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan in increased at least 3 weeks from the average 50% lethality time point of Mecp2tm1.1Bird hemizygotes

behavior/neurological
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

growth/size/body
N
• normal body weight unlike Mecp2tm1.1Bird hemizygotes




Genotype
MGI:5499846
cx7
Allelic
Composition
Mecp2tm1.1Bird/Y
Sum2M1Jus/Sum2+
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Sum2M1Jus mutation (0 available); any Sum2 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan in increased at least 3 weeks from the average 50% lethality time point of Mecp2tm1.1Bird hemizygotes

behavior/neurological
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

immune system
• unlike Mecp2tm1.1Bird hemizygotes
• unlike Mecp2tm1.1Bird hemizygotes

growth/size/body
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

vision/eye
• unlike Mecp2tm1.1Bird hemizygotes

integument
• unlike Mecp2tm1.1Bird hemizygotes




Genotype
MGI:5499847
cx8
Allelic
Composition
Mecp2tm1.1Bird/Y
Sum4M1Jus/Sum4+
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Sum4M1Jus mutation (0 available); any Sum4 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan in increased at least 3 weeks from the average 50% lethality time point of Mecp2tm1.1Bird hemizygotes

behavior/neurological
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 8 and 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes
• at 20 weeks but ameliorated compared to in Mecp2tm1.1Bird hemizygotes

craniofacial
• as in Mecp2tm1.1Bird hemizygotes

growth/size/body
N
• normal body weight unlike Mecp2tm1.1Bird hemizygotes

immune system
• unlike Mecp2tm1.1Bird hemizygotes
• unlike Mecp2tm1.1Bird hemizygotes

skeleton
• as in Mecp2tm1.1Bird hemizygotes

integument
• unlike Mecp2tm1.1Bird hemizygotes

vision/eye
• unlike Mecp2tm1.1Bird hemizygotes




Genotype
MGI:3624722
cx9
Allelic
Composition
Mbd2tm1Bh/Mbd2tm1Bh
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbd2tm1Bh mutation (0 available); any Mbd2 mutation (13 available)
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• exhibit the same mortality as single homozygous Mecp2 null mice

behavior/neurological
• exhibit the same onset of Rett-like syndrome symptoms and mortality as single homozygous Mecp2 null mice




Genotype
MGI:3817185
cx10
Allelic
Composition
Mecp2tm1.1Bird/Y
Tg(MECP2)1Hzo/0
Genetic
Background
involves: 129P2/OlaHsd * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Tg(MECP2)1Hzo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• premature death in male Mecp2-null mice at 10-11 weeks is rescued

behavior/neurological
N
• neurological abnormalities seen in single transgenic mutant are rescued by loss of Mecp2

nervous system
N
• amplitudes of EPSCs, RRP size, and mEPSC frequency, amplitude, and decay kinetics are normalized in double mutant brains compared to either single mutant brain
• glutamatergic synaptic density is normalized in double mutant brains compared to either single mutant brain




Genotype
MGI:5491040
cx11
Allelic
Composition
Mecp2tm1.1Bird/Y
Tg(MECP2*R270X/GFP)#Hzo/0
Genetic
Background
involves: 129P2/OlaHsd * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Tg(MECP2*R270X/GFP)#Hzo mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median lifespan of 85 days

growth/size/body
• at 8 weeks of age
• weight gain starts at 7 weeks of age and is more gradual than in mutant mice not carrying the transgene

nervous system
• brain weight peaks at 7 weeks (1 week earlier than in controls) then declines
• brain weight peaks at 7 weeks (1 week earlier than in controls) then declines
• 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
• 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

behavior/neurological
• at all ages tested and severity increases with age
• severity is reduced compared to mutant mice not carrying the transgene
• at all ages tested and severity increases with age
• severity is reduced compared to mutant mice not carrying the transgene

integument
• at 8 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:194039




Genotype
MGI:5491041
cx12
Allelic
Composition
Mecp2tm1.1Bird/Y
Tg(MECP2*G273X/GFP)#Hzo/0
Genetic
Background
involves: 129P2/OlaHsd * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
Tg(MECP2*G273X/GFP)#Hzo mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median lifespan of 201 days

growth/size/body
• begin to gain weight at 12 weeks of age reaching a maximum weight by 17 weeks of age

nervous system
• brain weight peaks at 7 weeks (1 week earlier than in controls) but does not decline unlike in mutant mice not carrying the transgene
• brain weight peaks at 7 weeks (1 week earlier than in controls) but does not decline unlike in mutant mice not carrying the transgene
• decrease in the number of ATRX containing foci at 9 weeks of age, later than in mutant mice not carrying the transgene
• decrease in the number of ATRX containing foci at 9 weeks of age, later than in mutant mice not carrying the transgene

behavior/neurological
• at all ages tested and severity increases with age
• severity is reduced compared to mutant mice not carrying the transgene
• at all ages tested and severity increases with age
• severity is reduced compared to mutant mice not carrying the transgene

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:194039




Genotype
MGI:3817230
ot13
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• no alteration in synaptic release probability is detected in neurons
• in vitro, density of synaptic markers is reduced by 39%
• in vivo, at 2 weeks postnatal, number of glutamatergic synapses is reduced by 19%; by 5 weeks, difference is no longer significant
• neurons exhibit a 41% reduction in RRP charge relative to wild-type
• 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
• at P40, hippocampal NOVA1 (neuro-oncological ventral antigen 1) mRNA and protein levels are significantly upregulated relative to those in wild-type controls
• evoked EPSC amplitudes show a 46% reduction compared to wild-type
• mEPSCs show a significant decrease in frequency compared to wild-type




Genotype
MGI:5499849
ot14
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• however, treatment with fluvastatin improves lifespan

behavior/neurological
• even in mice treated with fluvastatin
• however, treatment with fluvastatin or lovastatin improves coordination
• however, treatment with fluvastatin or lovastatin improves open-field activity

homeostasis/metabolism
• at P56
• however, statin treatment decreases serum cholesterol concentrations
• at P56, but not P70
• at P56
• however, treatment with fluvastatin or lovastatin improves liver lipid levels

craniofacial

growth/size/body

liver/biliary system
• at P56
• however, treatment with fluvastatin or lovastatin improves liver lipid levels

respiratory system
• even in mice treated with fluvastatin

skeleton

nervous system
• at P56, but not P70




Genotype
MGI:3624713
ot15
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• variable progression of symptoms leads to rapid weight loss and death at about 54 days of age

behavior/neurological
• most mutants develop hindlimb clasping after 7 weeks of age
• at 8 weeks of age, males show increased latency to start moving and to reach the wall of the open field apparatus
• exhibit reduced spontaneous movement between 3 and 8 weeks of age
• in the gait onset test, males take longer to exit a circle at 3 and 8 weeks of age
• 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
• develop a stiff, uncoordinated gait between 3 and 8 weeks of age (J:67910)
• males exhibit a greater front-base width overall and a larger hind-base width than wild-type mice by 3 weeks of age (J:165306)
• stride length is shorter than in wild-type mice at 8 weeks of age but not at 3 weeks of age

growth/size/body
• frequently exhibit uneven wearing of the teeth
• Background Sensitivity: mutants mated to C57BL/6 (mixed 129P2/OlaHsd and C57BL/6 background) mice are substantially underweight from 4 weeks with full penatrance
• variable progression of symptoms leads to rapid weight loss and death at about 54 days of age
• 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

respiratory system
• most mutants exhibit irregular breathing after 3-8 weeks of age

reproductive system
• testes of mutant males are always internal

craniofacial
• frequently exhibit misalignment of the jaws
• frequently exhibit uneven wearing of the teeth

endocrine/exocrine glands
• testes of mutant males are always internal

skeleton
• frequently exhibit misalignment of the jaws
• frequently exhibit uneven wearing of the teeth

hearing/vestibular/ear
• some mutants fail to respond to sound, although neither motor defects nor sensory defects are detected

homeostasis/metabolism
• 36% reduction in levels of norepinephrine within the vestibular nuclei
• 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
• mutants do not exhibit an increase in norepinephrine in the hippocampus from 3 to 8 weeks of age as seen in wild-type mice
• 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
• 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
• 5-HT turnover is increased in the prefrontal cortex and motor cortex
• 5-HT levels are decreased in the hippocampus at 8 weeks of age but not at 3 weeks

nervous system
• at 3 weeks of age, noradrenergic and serotonergic transmission is altered in the prefrontal and motor cortices
• during progression of disease, noradrenergic and serotonergic transmission is also altered in the hippocampus and cerebellum

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:67910 , J:165306




Genotype
MGI:3817273
ot16
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• male mutants become considerably heavier than littermates after 2 months, with noticeable weight differences at 9 weeks
• at 17 weeks, males 39.7 g vs 32.1 g in wild-type
• male mice become obese with increased weight of fat pads by 14-17 weeks

behavior/neurological
N
• visual placing reflex and response to a soft touch to the side of the face are normal relative to wild-type
• olfaction and motivation to eat were normal in mutants
• anxiety-related behaviors are similar to wild-type mice
• ability to grip a wire and remain suspended for 1 minute is normal in mutants
• subtle clasping is observed starting at 6 weeks of age
• 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

adipose tissue
• fat pads in 14-17 week old male mice weigh 2-3 times more than wild-type




Genotype
MGI:4999648
ot17
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• electrographic seizures are measured in cultured neurons from constitutive null mice

nervous system
• electrographic seizures are measured in cultured neurons from constitutive null mice
• non-seizure hyperexcitability discharges are observed in recordings from in cultured neurons from constitutive null mice
• electrographic seizures are measured in cultured neurons from constitutive null mice




Genotype
MGI:5491051
ot18
Allelic
Composition
Mecp2tm1.1Bird/Y
Genetic
Background
involves: 129P2/OlaHsd * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Bird mutation (1 available); any Mecp2 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median lifespan of 76 days

growth/size/body
• starting at 6 weeks of age and reaching a maximum weight by 8 weeks of age

nervous system
• brain weight peaks at 7 weeks (1 week earlier than in controls) then declines
• brain weight peaks at 7 weeks (1 week earlier than in controls) then declines
• 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
• 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

behavior/neurological
• at all ages tested and severity increases with age
• at all ages tested and severity increases with age

integument
• at 8 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:194039





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/28/2017
MGI 6.11
The Jackson Laboratory