Used to study neurodegeneration with brain iron accumulation 2a.
MGI Description
PHENOTYPE: As early as 3 weeks of age homozygotes show signs of sensory axonopathy (clasping reflex and ataxia) which progress to paraparesis and morbidity by 6 months of age. Large swollen axons (spheroids) are noted in the brainstem and spinal cord. Males fail tobreed, likely due to hindlimb muscle atrophy. [provided by MGI curators]