Mouse Genome Informatics
tg
    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