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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(NEFH)200Jpj
transgene insertion 200, Jean-Pierre Julien
MGI:2182068
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(NEFH)200Jpj/0
Tg(SOD1*G37R)29Dpr/0
involves: C3H/HeJ * C57BL/6 * C57BL/6J MGI:4947056
tg2
Tg(NEFH)200Jpj/Tg(NEFH)200Jpj Not Specified MGI:2183672
tg3
Tg(NEFH)200Jpj/0 involves: C57BL/6 MGI:4947054
tg4
Tg(NEFH)200Jpj/0 Not Specified MGI:2183678


Genotype
MGI:4947056
cx1
Allelic
Composition
Tg(NEFH)200Jpj/0
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6 * C57BL/6J
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 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

behavior/neurological
• delayed onset of paralysis
• occur shortly before death with a period of about 2 weeks duration

nervous system
• axonal atrophy
• prominent perikaryal neurofilamentous accumulations
• no massive axonal loss and cell death in the motor axons in one-year old mice
• axonal atrophy
• prominent perikaryal neurofilamentous accumulations
• no massive axonal loss and cell death in the sensory axons in one-year old mice




Genotype
MGI:2183672
tg2
Allelic
Composition
Tg(NEFH)200Jpj/Tg(NEFH)200Jpj
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• 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
• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs
• mice are described as displaying fine tremors by 3-4 months of age

muscle
• severely affected mice are unable to support their weight when grasping a pencil

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69180




Genotype
MGI:4947054
tg3
Allelic
Composition
Tg(NEFH)200Jpj/0
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• 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
• atrophy and slow degeneration of motor axons in old transgenic mice

behavior/neurological
• limb contraction reflexes during aging
• fine tremors during aging




Genotype
MGI:2183678
tg4
Allelic
Composition
Tg(NEFH)200Jpj/0
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• 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
• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs
• mice are described as displaying fine tremors by 3-4 months of age

muscle
• severely affected mice are unable to support their weight when grasping a pencil

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69180





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last database update
05/17/2016
MGI 6.03
The Jackson Laboratory