Phenotypes associated with this allele
Allelic Composition |
Mecp2tm1.1Jae/Mecp2+
|
|
Genetic Background |
involves: 129S4/SvJae * BALB/c * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
|
|
|
behavior/neurological
|
• females appear normal for the first 4 months but show ataxic gait at later ages
|
|
• females appear normal for the first 4 months but show hypoactivity at later ages
|
growth/size/body
|
• females appear normal for the first 4 months but gain weight at later ages
|
respiratory system
|
• mutants at 10 weeks of age exhibit an abnormally high breathing frequency associated with marked decreases in expiratory time and total breath duration and a small but significant decrease in inspiratory time
• treatment with LM22A-4, a small molecule BDNF loop domain mimetic that acts as a selective TrkB agonist, improves the respiratory function of mutants
|
|
• breathing dysfunction is characterized by increased frequency due to periods of tachypnea and increased apneas
|
|
• 20% of mutants exhibit apneas at 8 weeks of age and by 12 weeks of age, 50% of mutants exhibit apneas
• number of apneas increases between 8 and 12 weeks of age in mutants
|
Allelic Composition |
Mecp2tm1.1Jae/Mecp2+
|
|
Genetic Background |
involves: 129S4/SvJae * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
|
|
|
behavior/neurological
growth/size/body
|
• mutants are severely underweight from 4-5 weeks of age
|
nervous system
|
• 8 - 13 week old mutants display a 14-18% reduction in brain weight
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Col1a1tm8(CAG-BDNF)Jae mutation
(0 available);
any
Col1a1 mutation
(160 available)
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
Tg(Camk2a-cre)93Kln mutation
(1 available)
|
|
|
mortality/aging
|
• life span is significantly longer than in Mecp2 single hemizygous mice
|
behavior/neurological
N |
• overexpression of BDNF improves locomotor function compared to Mecp2 single hemizygous mice at 6 weeks of age
|
nervous system
|
• decreased compared to wild-type littermates but modestly increased compared to Mecp2 single hemizygous mice
|
|
• firing rate is increased compared to Mecp2 single hemizygous mice
|
mortality/aging
|
• life span is shorter than in Mecp2 single hemizygous mice but longer than in mutant mice homozygous for the Bdnf allele
|
mortality/aging
|
• life span is shorter than in Mecp2 single homozygous mice
|
behavior/neurological
|
• dramatic reduction in wheel running activity at 4 weeks of age
|
nervous system
|
• at P30 the firing rate of layer 5 pyramidal neurons of the somatosensory cortex is reduced compared to controls
|
behavior/neurological
N |
• mutant activity as measured by exploratory response and total nocturnal activity does not differ from wild-type mice
|
growth/size/body
N |
• mutants display normal body weight
|
nervous system
N |
• mutants display normal brain weight
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
|
|
|
homeostasis/metabolism
liver/biliary system
nervous system
Allelic Composition |
Mecp2tm1.1Jae/Y
|
|
Genetic Background |
involves: 129S4/SvJae * BALB/c * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
|
|
|
mortality/aging
|
• most die at about 10 weeks of age without obvious correlation between physical deterioration and time of death
|
behavior/neurological
|
• appear healthy for the first few weeks of age but develop abnormal behavior, such as nervousness, at 5 weeks of age
|
|
• exhibit body trembling at 5 weeks of age
• at late stages of disease, mutants tremble when handled
|
|
• seen at late stages of disease
|
nervous system
|
• neurons in the CA2 region are 15-25% smaller than in controls at 9 weeks of age
|
|
• cell bodies and nuclei or neurons in sections of hippocampus, cerebral cortex, and cerebellum are smaller in size and more densely packed
|
growth/size/body
|
• most exhibit signs of physical deterioration by 8 weeks of age and often begin to lose weight at late stages of disease
|
|
• 8 of 17 become overweight and obese at 40-60 days of age
|
homeostasis/metabolism
respiratory system
|
• occasionally show heavy breathing at 5 weeks of age
|
Allelic Composition |
Mecp2tm1.1Jae/Y
|
|
Genetic Background |
involves: 129S4/SvJae * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1.1Jae mutation
(2 available);
any
Mecp2 mutation
(38 available)
|
|
|
mortality/aging
nervous system
|
• decrease size of CA2 neurons
|
|
• decreased size of the olfactory glomeruli
|
|
• at P30 the firing rate of layer 5 pyramidal neurons of the somatosensory cortex is reduced compared to controls
|
|
• 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
|
• develops at 6 weeks of age
|
|
• impaired motor learning on a rotarod
|
|
• in an elevated zero maze
|
|
• with splaying hind limbs upon movement
|