Phenotypes associated with this allele
Allelic Composition |
Fgf6tm1Thbr/Fgf6tm1Thbr
|
|
Genetic Background |
either: (involves: 129S4/SvJae-Fgf6tm1Thbr) or (involves: 129S4/SvJae * C57BL/6) |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
|
|
|
craniofacial
N |
• maxilla and mandible normal
|
muscle
|
• defective regeneration of skeletal muscle after crush-injury
• apparent 4 days after injury and strikingly abnormal after 10-14 days when controls have completely recovered
• mononuclear cells and remnants of degenerating myotubules persist after 2 weeks
• collagen deposits, evidence of fibrosis at 2 weeks and persisting to 3 weeks after injury
|
cellular
|
• myogenic cells from mutants show impaired migration both distally and proximally to the injury site from the injection site when transplanted into wild-type mice (in which the tibialis anterior muscle had been injured and was undergoing regeneration)
|
growth/size/body
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
Myod1tm1Jae mutation
(2 available);
any
Myod1 mutation
(23 available)
|
|
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf5tm1Tzi mutation
(0 available);
any
Fgf5 mutation
(14 available)
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
Fgf7tm1Efu mutation
(1 available);
any
Fgf7 mutation
(17 available)
|
|
|
integument
|
• hair length is increased compared to Fgf5tm1Tzi homozygotes
|
|
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)
|
|
|
muscle
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
|
|
• 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
|
cellular
|
• 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
|
|
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)
|
|
|
muscle
N |
• growth and differentiation kinetics of myogenic cells are comparable to wild-type
|
|
• pale areas in muscle contain extensive connective tissue
|
|
• 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
|
|
• numerous necrotic fibers are present
|
|
• in pale areas of muscles, remaining myotubes show large variation in caliber size
|
|
• myotubes have slightly reduced percentage of satellite cells with respect to myotube nuclei in wild-type (3.05% vs 3.56%)
|
|
• severe abnormalities are observed
|
|
• mutants display palpable stiffness of the musculature, most evident in pelvic and shoulder girdle, at up to 6 months of age
|
skeleton
|
• up to 6 months of age, mice do not show clinical signs of severe dystrophy except for dorsal-ventral curvature of the spine
|
cellular
|
• numerous necrotic fibers are present
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf5tm1Tzi mutation
(0 available);
any
Fgf5 mutation
(14 available)
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
|
|
|
integument
|
• hair length is increased compared to Fgf5tm1Tzi homozygotes
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation
(30 available);
any
Dmd mutation
(153 available)
Fgf6tm1Thbr mutation
(0 available);
any
Fgf6 mutation
(7 available)
|
|
|
skeleton
|
• dorso-ventral curvature of the spine
|
muscle
N |
• no clinical signs of severe dystrophy for up to 6 months
|
|
• back and body wall musculature show changes similar to diaphragm but less uniformly and more focally distributed
|
|
• muscle changes similar to diaphragm but less uniformly and more focally distributed
|
|
• pronounced increase in collagen
• increase in mononuclear cells
• unusually small myotubes with very few centrally located nuclei
|
|
• changes are not enhanced compared to Dmd mutants
|
|
• severe myopathy observed in diaphragm, limb muscles, and back muscles
|