Phenotypes associated with this allele

• Allele Symbol: Mecp2^tm1.1Joez
• Allele Name: targeted mutation 1.1, Zhaolan Zhou
• Allele ID: MGI:5310727
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Mecp2^tm1.1Joez/Mecp2^+	B6.129-Mecp2^tm1.1Joez	MGI:5310738
cx2	Mecp2^tm1.1Joez/Y Tg(Thy1-EGFP)#Jrs/0	involves: 129 * C57BL/6 * C57BL/6J * CBA	MGI:5310737
ot3	Mecp2^tm1.1Joez/Y	B6.129-Mecp2^tm1.1Joez	MGI:5310735

Genotype
MGI:5310738

ht1

• Allelic Composition: Mecp2^tm1.1Joez/Mecp2⁺
• Genetic Background: B6.129-Mecp2^tm1.1Joez

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

mortality/aging

mortality/aging ( J:181311 )

♀N • mice exhibit normal survival

behavior/neurological

abnormal behavior ( J:181311 )

♀ • mice exhibit RTT-like symptoms after 17 weeks of age

decreased locomotor activity ( J:181311 )

♀ • at 20, but not 12, weeks of age

growth/size/body

increased body weight ( J:181311 )

♀

Genotype
MGI:5310737

cx2

• Allelic Composition: Mecp2^tm1.1Joez/Y; Tg(Thy1-EGFP)#Jrs/0
• Genetic Background: involves: 129 * C57BL/6 * C57BL/6J * CBA

nervous system

abnormal hippocampus pyramidal cell morphology ( J:181311 )

♂ • at P30 and P90, hippocampal CA1 pyramidal neurons exhibit decreased soma size compared with control cells

Genotype
MGI:5310735

ot3

• Allelic Composition: Mecp2^tm1.1Joez/Y
• Genetic Background: B6.129-Mecp2^tm1.1Joez

mortality/aging

premature death ( J:181311 )

♂ • 50% of mice die by 16 weeks of age

behavior/neurological

abnormal behavior ( J:181311 )

♂ • mice exhibit RTT-like symptoms after 5 weeks of age

impaired contextual conditioning behavior ( J:181311 )

♂

impaired cued conditioning behavior ( J:181311 )

♂

abnormal motor learning ( J:181311 )

♂ • impaired motor learning on a rotarod

decreased anxiety-related response ( J:181311 )

♂ • in an elevated zero maze

limb grasping ( J:181311 )

♂ • at 13 weeks of age

impaired coordination ( J:181311 )

♂ • on a rotarod

♂ • not as severe as in Mecp2^tm1.1Jae hemizygotes

akinesia ( J:181311 )

♂

abnormal gait ( J:181311 )

♂ • with splaying hind limbs upon movement

decreased locomotor activity ( J:181311 )

♂ • at 11, but not 3, weeks of age

♂ • not as severe as in Mecp2^tm1.1Jae hemizygotes

seizures ( J:181311 )

♂ • occasionally after 5 weeks of age

nervous system

seizures ( J:181311 )

♂ • occasionally after 5 weeks of age

decreased brain weight ( J:181311 )

♂ • at P30 and P90

abnormal brain wave pattern ( J:181311 )

♂ • at P30, mice exhibit reduction in event-related power in delta, sigma and alpha low-frequencies compared with wild-type mice

♂ • at P30, mice exhibit less of an increase in phase-locking factor at delta and high gamma frequencies compared with wild-type mice

♂ • at P90, awake mice exhibit increased high-gamma frequency oscillation power compared with wild-type mice

♂ • at P90, mice exhibit an increase in latency of P1, N1 and P2 peaks and reduction in the amplitudes of the N1 and P2 peaks of event-related potential compared with wild-type mice

♂ • at P90, mice exhibit attenuated event-related power in both low- and high-frequency oscillation compared with wild-type mice

♂ • at P90, mice exhibit less of an increase in phase-locking factor at all frequencies compared with wild-type mice

♂ • however, mice exhibit normal power and event-related potential at P30

abnormal brain alpha wave pattern ( J:181311 )

♂

abnormal brain delta wave pattern ( J:181311 )

♂

growth/size/body

decreased body weight ( J:181311 )

♂ • between 4 and 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:181311