Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(NEFH)200Jpj mutation
(0 available)
Tg(SOD1*G37R)29Dpr mutation
(1 available)
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mortality/aging
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• extended mean longevity by about 6 months, compared with Tg(SOD1*G37R)29Dpr/0 mice
• all the mice are still alive after 1 year
• average lifespan is 15.8 months
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behavior/neurological
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• delayed onset of paralysis
• occur shortly before death with a period of about 2 weeks duration
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nervous system
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• prominent perikaryal neurofilamentous accumulations
• axonal atrophy
• no massive axonal loss and cell death in the motor axons in one-year old mice
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• axonal atrophy
• prominent perikaryal neurofilamentous accumulations
• no massive axonal loss and cell death in the sensory axons in one-year old mice
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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nervous system
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• spinal cord sections revealed many abnormalities in motor neurons of the anterior horn and the dorsal root ganglia, including swelling of perikaraya and proximal axons
• immunofluorescence staining demonstrated that the swelling was the result of heteropolymerization of multiple neurofilament subunits
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• distal axons of the sciatic nerve appear shrunken in caliber with thick myelin sheaths
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behavior/neurological
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• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs
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• mice are described as displaying fine tremors by 3-4 months of age
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muscle
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• severely affected mice are unable to support their weight when grasping a pencil
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Allelic
Composition |
Tg(NEFH)200Jpj/0
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Genetic
Background |
involves: C57BL/6 |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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nervous system
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• neurofilament accumulations in lower motor neurons
• more prominent neurofilamentous swellings in spinal motor neurons and more severe atrophy of ventral and dorsal root axons than Tg(NEFH)398Jpj/0 mice
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• atrophy and slow degeneration of motor axons in old transgenic mice
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behavior/neurological
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• limb contraction reflexes during aging
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• fine tremors during aging
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
|
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nervous system
|
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• spinal cord sections revealed many abnormalities in motor neurons of the anterior horn and the dorsal root ganglia, including swelling of perikaraya and proximal axons
• immunofluorescence staining demonstrated that the swelling was the result of heteropolymerization of multiple neurofilament subunits
|
|
|
• distal axons of the sciatic nerve appear shrunken in caliber with thick myelin sheaths
|
behavior/neurological
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• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs
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• mice are described as displaying fine tremors by 3-4 months of age
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muscle
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• severely affected mice are unable to support their weight when grasping a pencil
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Analysis Tools