Phenotypes associated with this allele
Allelic
Composition |
Utrntm1Ked/Utrntm1Ked
|
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Genetic
Background |
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA |
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Utrntm1Ked mutation
(9 available);
any
Utrn mutation
(306 available)
|
|
|
nervous system
|
|
• decrease in junctional folding at the postsynaptic membrane
• reduction in number of acetylcholine receptors in each junction
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|
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• the amplitude of miniature endplate currents is reduced by 20% in the extensor digitorum longus muscle, however other features of neuromuscular transmission are normal
|
muscle
|
N |
• no indication of muscle weakness or degeneration of cardiac or skeletal muscle
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renal/urinary system
|
N |
• normal kidney and function
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respiratory system
|
N |
• no signs of abnormal breathing and the lung appears normal
|
muscle
 |
• authors state that mice develop the same skeletal muscle abnormalities as observed in Dmdmdx Utrntm1Ked mice
|
nervous system
|
N |
• unlike in Dmdmdx Utrntm1Ked mice, brain morphology is normal
|
respiratory system
|
N |
• unlike in Dmdmdx Utrntm1Ked mice, lung morphology is normal
|
liver/biliary system
|
N |
• liver morphology is normal
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renal/urinary system
|
N |
• kidney morphology is normal
|
reproductive system
|
N |
• ovary morphology is normal
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muscle
 |
• authors state that mice develop the same skeletal muscle abnormalities as observed in Dmdmdx Utrntm1Ked mice
|
nervous system
|
N |
• unlike in Dmdmdx Utrntm1Ked mice, brain morphology is normal
|
respiratory system
|
N |
• unlike in Dmdmdx Utrntm1Ked mice, lung morphology is normal
|
liver/biliary system
|
N |
• liver morphology is normal
|
renal/urinary system
|
N |
• kidney morphology is normal
|
reproductive system
|
N |
• testis morphology is normal
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mortality/aging
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• all succumb to premature death by 20 weeks of age
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behavior/neurological
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• slack posture by 4 to 6 weeks of age
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• decrease in mobility and abnormal field behavior by 4 to 6 weeks of age
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growth/size/body
muscle
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• muscle fibers are irregularly shaped and sized, many with centralized nuclei and some lymphocyte infiltration
• some fibers contain fat or appear to be replaced by adipose cells
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• muscular dystrophy begins at 6 days of age and becomes severe by 10 weeks of age, earlier than in single Dmd mutants
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respiratory system
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• exhibit abnormal breathing by 4 to 6 weeks of age
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skeleton
nervous system
|
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• postsynaptic endplate regions exhibit a virtual absence of junctional folding at the postsynaptic membrane
• about 40% decrease in acetylcholine receptors at the synapse
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limbs/digits/tail
|
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• abnormally postured hindlimbs with joint contractures
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cellular
Analysis Tools