Phenotypes associated with this allele

• Allele Symbol: Tg(NEFH)200Jpj
• Allele Name: transgene insertion 200, Jean-Pierre Julien
• Allele ID: 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

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:69179 )

• 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

paralysis ( J:69179 )

• delayed onset of paralysis

• occur shortly before death with a period of about 2 weeks duration

nervous system

motor neuron degeneration ( J:69179 )

• axonal atrophy

• prominent perikaryal neurofilamentous accumulations

• no massive axonal loss and cell death in the motor axons in one-year old mice

decreased sensory neuron number ( J:69179 )

• 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

nervous system

abnormal motor neuron morphology ( J:69180 )

• 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

abnormal axon morphology ( J:69180 )

• distal axons of the sciatic nerve appear shrunken in caliber with thick myelin sheaths

behavior/neurological

limb grasping ( J:69180 )

• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs

tremors ( J:69180 )

• mice are described as displaying fine tremors by 3-4 months of age

muscle

progressive muscle weakness ( J:69180 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:69180

Genotype
MGI:4947054

tg3

• Allelic Composition: Tg(NEFH)200Jpj/0
• Genetic Background: involves: C57BL/6

nervous system

abnormal motor neuron morphology ( J:69179 )

• 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

motor neuron degeneration ( J:69179 )

• atrophy and slow degeneration of motor axons in old transgenic mice

behavior/neurological

limb grasping ( J:69179 )

• limb contraction reflexes during aging

tremors ( J:69179 )

• fine tremors during aging

Genotype
MGI:2183678

tg4

• Allelic Composition: Tg(NEFH)200Jpj/0
• Genetic Background: Not Specified

nervous system

abnormal motor neuron morphology ( J:69180 )

• 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

abnormal axon morphology ( J:69180 )

• distal axons of the sciatic nerve appear shrunken in caliber with thick myelin sheaths

behavior/neurological

limb grasping ( J:69180 )

• when lifted by the tail, mice contract their forelimbs and hindlimbs, compared to control mice that extend their legs

tremors ( J:69180 )

• mice are described as displaying fine tremors by 3-4 months of age

muscle

progressive muscle weakness ( J:69180 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:69180