Phenotypes associated with this allele

• Allele Symbol: Tg(NFH)120Jpj
• Allele Name: transgene insertion 120, Jean-Pierre Julien
• Allele ID: MGI:2182067
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(NFH)120Jpj/Tg(NFH)120Jpj	Not Specified	MGI:2183662
tg2	Tg(NFH)120Jpj/0	Not Specified	MGI:2183668

Genotype
MGI:2183662

tg1

• Allelic Composition: Tg(NFH)120Jpj/Tg(NFH)120Jpj
• Genetic Background: Not Specified

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

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 6 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:2183668

tg2

• Allelic Composition: Tg(NFH)120Jpj/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 6 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