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Phenotypes Associated with This Genotype
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
gnd mutation (1 available); any gnd mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• smaller size is identifiable at 2.5 weeks of age

nervous system
• dystrophic axons are present in large numbers at all levels of the spinal cord in all white matter funiculi and in central grey matter and are commonly found throughout the brain stem, including nucleus gracilis, cerebellar peduncles and deep cerebellar nuclei, red nucleus, and substantia nigra and in the optic nerve and tract, corpus callosum, rostral commissure and fornix of the forebrain

• reduced ability to stand on either fore or hind limbs when tested for motor deficits
• at 3 months of age, have a humped back
• progressively develop a shaky gait and tend to move the distal hind limbs only below the hock
• at 3 months of age, the hip joint exhibits little flexion and forelimbs tend to splay out to the side
• hind limbs are paralyzed by 8 months of age and are dragged along behind, however never exhibit spasticity
• only rarely do mutants breed

• exhibit a dull hair coat by 2.5 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
neurodegeneration with brain iron accumulation 2a DOID:0110735 OMIM:256600

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.12
The Jackson Laboratory