Phenotypes Associated with This Genotype

Genotype
MGI:5306241

• Allelic Composition: Mecp2^tm1.1Jae/Y
• Genetic Background: involves: 129S4/SvJae * C57BL/6

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

mortality/aging

premature death ( J:106973 )

♂

nervous system

decreased brain weight ( J:106973 )

♂

abnormal hippocampus CA2 region morphology ( J:106973 )

♂ • decrease size of CA2 neurons

abnormal olfactory bulb morphology ( J:106973 )

♂ • decreased size of the olfactory glomeruli

abnormal action potential ( J:106973 )

♂ • at P30 the firing rate of layer 5 pyramidal neurons of the somatosensory cortex is reduced compared to controls

abnormal brain wave pattern ( J:181311 )

♂ • 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 event related phase-locking factor compared with wild-type mice

behavior/neurological

lethargy ( J:106973 )

♂ • develops at 6 weeks of age

abnormal motor learning ( J:181311 )

♂ • impaired motor learning on a rotarod

decreased anxiety-related response ( J:181311 )

♂ • in an elevated zero maze

impaired coordination ( J:181311 )

♂ • on a rotarod

abnormal gait ( J:181311 )

♂ • with splaying hind limbs upon movement

decreased locomotor activity ( J:181311 )

♂

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Rett syndrome		DOID:1206	OMIM:312750 OMIM:613454	J:106973