Mouse Genome Informatics
cx1
    Tg(NEFH)200Jpj/0
Tg(SOD1*G37R)29Dpr/0

involves: C3H/HeJ * C57BL/6 * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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


Mouse Genome Informatics
tg2
    Tg(NEFH)200Jpj/Tg(NEFH)200Jpj
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69180


Mouse Genome Informatics
tg3
    Tg(NEFH)200Jpj/0
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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


Mouse Genome Informatics
tg4
    Tg(NEFH)200Jpj/0
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69180