Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf2tm1Zllr mutation
(0 available);
any
Fgf2 mutation
(17 available)
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muscle
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N |
• mice do not show any changes in the musculature compared to double and triple mutants carrying the Dmdmdx allele; no dystrophic muscle fibers are detected
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf2tm1Zllr mutation
(0 available);
any
Fgf2 mutation
(17 available)
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nervous system
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• in newborn homozygotes, a fraction of neuronal progenitors fail to colonize their target cortical layers III and II during cerebral cortex development; as a result, a greater number (~17%) of BrdU-labeled cells remain in the deeper cortical layers (VI-IV) and the corpus callosum relative to wild-type mice (~8.8%)
• this defect is variable, as many more BrdU-positive cells (25-36%) are found in the corpus callosum and deep layers of severely affected homozygotes, whereas a few others are indistinguishable from wild-type
• adult homozygotes display varying degrees of ectopic parvalbumin-positive neurons in the hippocampal commissure
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• adult homozygotes display varying degrees of ectopic parvalbumin-positive neurons in the hippocampal commissure located beneath the cerebral cortex
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• at birth, homozygotes show defects in organization and differentiation of the cerebral cortex
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• at birth, only a few differentiated, large pyramidal neurons are detected in the cerebral cortex
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• at birth, all differentiating cortical layers appear compressed and less distinct
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• an average reduction of ~25% in parvalbumin-positive neurons is observed in all cortical layers
• however, no differences in the distribution of astroglial cells are observed
• no significant differences are noted in cell survival in either embryonic (E16), newborn or adult cerebral cortex, as shown by TUNEL analysis
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• at birth, the thickness of the cerebral cortex is reduced by ~10%
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• adult homozygotes show neuronal abnormalities throughout the mantle layer of the cervical spinal cord, with only a few differentiated large neurons while all other neural cell types appear normal
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• adult homozygotes respond normally to chronic angiotensin II infusion but show an impaired baroreceptor reflex
• in response to an acute vasodilatory stimulus (isoproterenol), all homozygotes, but no wild-type mice, display a significant drop in blood pressure along with an impaired increase in heart rates, indicating autonomic dysfunction
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cardiovascular system
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• on average, the blood pressure of adult homozygotes is reduced by ~10 mmHg relative to wild-type littermates
• however, no differences in baseline plasma renin values, heart rates or body weights are observed
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behavior/neurological
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N |
• homozygotes do NOT develop seizures or reeler-like phenotypes
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hearing/vestibular/ear
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N |
• homozygotes exhibit normal inner ear morphogenesis with no detectable abnormalities at the adult stage
• in addition, homozygotes show no significant differences in hearing thresholds before and after noise-induced cochlear damage relative to wild-type littermates
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cellular
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• in newborn homozygotes, a fraction of neuronal progenitors fail to colonize their target cortical layers III and II during cerebral cortex development; as a result, a greater number (~17%) of BrdU-labeled cells remain in the deeper cortical layers (VI-IV) and the corpus callosum relative to wild-type mice (~8.8%)
• this defect is variable, as many more BrdU-positive cells (25-36%) are found in the corpus callosum and deep layers of severely affected homozygotes, whereas a few others are indistinguishable from wild-type
• adult homozygotes display varying degrees of ectopic parvalbumin-positive neurons in the hippocampal commissure
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf2tm1Zllr mutation
(0 available);
any
Fgf2 mutation
(17 available)
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
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muscle
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N |
• mice do not show any changes in the musculature compared to double and triple mutants carrying the Dmdmdx allele; no dystrophic muscle fibers are detected
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• basal level of migration of mutant myoblasts in culture is reduced by ~one-third relative to wild-type; migratory response to stimulation by Fgf2 and Fgf6 is significantly increased but does not reach level of stimulated wild-type myoblasts
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cellular
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• basal level of migration of mutant myoblasts in culture is reduced by ~one-third relative to wild-type; migratory response to stimulation by Fgf2 and Fgf6 is significantly increased but does not reach level of stimulated wild-type myoblasts
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation
(30 available);
any
Dmd mutation
(153 available)
Fgf2tm1Zllr mutation
(0 available);
any
Fgf2 mutation
(17 available)
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
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muscle
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N |
• growth and differentiation kinetics of myogenic cells are comparable to wild-type
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• pale areas in muscle contain extensive connective tissue
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• when mice are injected with the diazo dye EBD, dye is taken up by damaged muscle fibers to varying degrees, but preferentially into the hindlimb; uptake occurs because of focal breakdown of plasmalemma which is an early event in necrosis
• some muscles are stained entirely indicating massive damage to the muscle fibers of the diaphragm and gluteus maximus whereas muscles in wild-type mice do not take up dye
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• numerous necrotic fibers are present
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• in pale areas of muscles, remaining myotubes show large variation in caliber size
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• myotubes have slightly reduced percentage of satellite cells with respect to myotube nuclei in wild-type (3.05% vs 3.56%)
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• severe abnormalities are observed
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• mutants display palpable stiffness of the musculature, most evident in pelvic and shoulder girdle, at up to 6 months of age
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skeleton
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• up to 6 months of age, mice do not show clinical signs of severe dystrophy except for dorsal-ventral curvature of the spine
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cellular
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• numerous necrotic fibers are present
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation
(30 available);
any
Dmd mutation
(153 available)
Fgf2tm1Zllr mutation
(0 available);
any
Fgf2 mutation
(17 available)
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muscle
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• mutants show dystrophic changes to the muscle fibers, although less severely than Fgf2/Fgf6/Dmd triple mutants
• changes are not enhanced compared to Dmd mutants
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Analysis Tools
