All Phenotypes Tg(MECP2)1Hzo MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Mecp2^tm1.1Bird/Y Tg(MECP2)1Hzo/0	involves: 129P2/OlaHsd * FVB	MGI:3817185
cx2	Crh^tm1Maj/Crh⁺ Tg(MECP2)1Hzo/0	involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * FVB/N	MGI:5314414
cx3	Oprm1^tm1Kff/Oprm1⁺ Tg(MECP2)1Hzo/0	involves: 129S2/SvPas * C57BL/6 * FVB/N	MGI:5314413
cx4	Crhr1^tm1Klee/Crhr1⁺ Tg(MECP2)1Hzo/0	involves: 129S4/SvJae * 129S6/SvEv * C57BL/6 * FVB/N	MGI:5314415
tg5	Tg(MECP2)1Hzo/0	either: (FVB/N x 129S6/SvEvTac)F1 or (FVB/N x C57BL/6J)F1	MGI:5314411
tg6	Tg(MECP2)1Hzo/0	FVB-Tg(MECP2)1Hzo/J	MGI:6305084
tg7	Tg(MECP2)1Hzo/0	involves: FVB	MGI:3817183

Allelic Composition	Mecp2^tm1.1Bird/Y Tg(MECP2)1Hzo/0
Genetic Background	involves: 129P2/OlaHsd * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2^tm1.1Bird mutation (1 available); any Mecp2 mutation (41 available)
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

mortality/aging ( J:94407 )

N	• premature death in male Mecp2-null mice at 10-11 weeks is rescued

behavior/neurological

behavior/neurological phenotype ( J:94407 )

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

nervous system

nervous system phenotype ( J:126964 )

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

Allelic Composition	Crh^tm1Maj/Crh⁺ Tg(MECP2)1Hzo/0
Genetic Background	involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Crh^tm1Maj mutation (2 available); any Crh mutation (42 available)
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

abnormal anxiety-related response ( J:181213 )

• anxiety-like behavior is reduced compared to single Tg(MECP2)1Hzo hemizygous mice, with double mutants being less anxious in the elevated plus maze and the light/dark box

homeostasis/metabolism

abnormal corticosterone level ( J:181213 )

• mutants exhibit a decrease in stress-induced serum corticosterone levels from that seen in single Tg(MECP2)1Hzo hemizygous mice

Allelic Composition	Oprm1^tm1Kff/Oprm1⁺ Tg(MECP2)1Hzo/0
Genetic Background	involves: 129S2/SvPas * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Oprm1^tm1Kff mutation (1 available); any Oprm1 mutation (54 available)
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

increased anxiety-related response ( J:181213 )

• double mutants exhibit a similar level of anxiety-like behavior in the elevated plus maze and light/dark box as single Tg(MECP2)1Hzo hemizygous mice

• however, double mutants exhibit improved social behavior deficits compared to single Tg(MECP2)1Hzo mice, spending more time investigating both familiar and novel partners

Allelic Composition	Crhr1^tm1Klee/Crhr1⁺ Tg(MECP2)1Hzo/0
Genetic Background	involves: 129S4/SvJae * 129S6/SvEv * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Crhr1^tm1Klee mutation (1 available); any Crhr1 mutation (28 available)
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

abnormal anxiety-related response ( J:181213 )

• anxiety-like behavior is reduced compared to single Tg(MECP2)1Hzo hemizygous mice, with double mutants being less anxious in the elevated plus maze and the light/dark box

Allelic Composition	Tg(MECP2)1Hzo/0
Genetic Background	either: (FVB/N x 129S6/SvEvTac)F1 or (FVB/N x C57BL/6J)F1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

increased anxiety-related response ( J:181213 )

• mutants exhibit anxiety-like behavior in the elevated plus maze

• mutants spend less time in the lit compartment of the light/dark box compared to wild-type mice indicating anxiety-like behavior

abnormal response to novel object ( J:323615 )

• 70-day old mice show reduced curiosity in exploration of a novel object than wild-type mice

• treatment of mice with miR-592 increases curiosity in object exploration to wild-type levels

abnormal social investigation ( J:181213 )

• mutants are less interested in their familiar or novel partner mice in the partition test for social interaction and recognition

• in the three-chamber test for sociability, mutants show a deficit in social approach behavior toward novel partner mice, without a deficit in interest toward a novel object or a deficit in activity or preference for either chamber

homeostasis/metabolism

abnormal corticosterone level ( J:181213 )

• stress-induced corticosterone levels are higher in mutants than wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
syndromic X-linked intellectual disability Lubs type		DOID:0060799	OMIM:300260	J:181213

Allelic Composition	Tg(MECP2)1Hzo/0
Genetic Background	FVB-Tg(MECP2)1Hzo/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(MECP2)1Hzo mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

vision/eye

abnormal visual evoked potential ( J:253720 )

• V1 neurons show age-dependent change in firing rate, with higher evoked responses at 8 weeks but lower at 14 weeks and lower firing rates to low and high spatial frequencies in 14 week old V1 neurons

abnormal visual acuity ( J:253720 )

• primary visual cortex (V1) neurons show a preference for higher spatial frequencies at 8 and 14 weeks of age indicating higher visual acuity

• in a two-alternative forced choice visual detection task, mice exhibit higher behavioral performance in spatial frequency detection

abnormal visual contrast sensitivity ( J:253720 )

• V1 neurons show age-dependent (at 8 weeks but not 14 weeks of age) change in contrast sensitivity, indicating higher contrast sensitivity

• behavioral performance in visual detection of stimuli that varies in contrast shows improved performance in detecting low contrasts

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
syndromic X-linked intellectual disability Lubs type		DOID:0060799	OMIM:300260	J:253720

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

premature death ( J:94407 )

• 30% of mice die between 20 weeks and 1 year of age; mice surviving past 1 year of age have a normal lifespan

nervous system

nervous system phenotype ( J:126964 )

N	• no alteration in synaptic release probability is detected in neurons

clonic seizures ( J:94407 )

• visually observed as early as 20 weeks of age, with increased frequency by 50 weeks

increased CNS synapse formation ( J:126964 )

• in vitro, density of synaptic markers is increased by 60%

• in vivo, at 2 weeks postnatal, number of glutamatergic synapses is increased by 80%; by 5 weeks, difference is much smaller but is still slightly significant

abnormal synaptic vesicle number ( J:126964 )

• neurons exhibit a 104% enhancement in RRP charge relative to wild-type

increased excitatory postsynaptic current amplitude ( J:126964 )

• evoked EPSC amplitudes show a 116% enhancement compared to wild-type

enhanced long-term potentiation ( J:94407 )

• robust enhancement is observed in mutants

abnormal miniature excitatory postsynaptic currents ( J:126964 )

• mEPSCs show a significant increase in frequency compared to wild-type

enhanced paired-pulse facilitation ( J:94407 )

• PPF is greatly enhanced in transgenic mice

behavior/neurological

abnormal contextual conditioning behavior ( J:94407 )

• in conditioned fear analysis, mutants and wild-type show similar results in cued fear conditioning, but in contextual conditioning, mutants show elevated freezing behavior indicating greater hippocampal learning

increased aggression ( J:94407 )

• increased propensity to bite

decreased anxiety-related response ( J:94407 )

• mice show lower anxiety evidenced by increased vertical activity in open field test

limb grasping ( J:94407 )

• forepaw clasping when lifted by tail

abnormal motor coordination/balance ( J:94407 )

• in dowel test, most animals remain inactive on wooden rod the entire trial period

enhanced coordination ( J:94407 )

• at 10 weeks of age, transgenics perform better on a rotating rod test on day 2 of training relative to wild-type; upon retesting at 20 weeks, mutants perform significantly better than wild-type

increased vertical activity ( J:94407 )

• mice show increased vertical activity compared to controls at 10 and 20 weeks

decreased locomotor activity ( J:94407 )

• in dowel test, transgenic animals display significantly reduced movement and walk off the length of an elevated dowel fewer times than wild-type

• characterized by freezing behavior

clonic seizures ( J:94407 )

• visually observed as early as 20 weeks of age, with increased frequency by 50 weeks

skeleton

kyphosis ( J:94407 )

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