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Phenotypes Associated with This Genotype
Not Specified
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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

• 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 6 months of age

• 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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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.07
The Jackson Laboratory