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PMID:3783318

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Citation

Messer, A and Flaherty, L (1986) Autosomal dominance in a late-onset motor neuron disease in the mouse. J. Neurogenet. 3:345-55

Abstract

A late-onset neurological disease has been identified in a substrain of C57Bl/6 mice. The disorder is characterized by hindlimb weakness and ataxia starting at 5-11 months of age, progressing to severe spastic paralysis of all limbs, with premature death. Histopathology reveals degeneration of upper and lower motoneurons. Both sexes are affected; the mice are fertile, although breeding efficiency is reduced. In outcrosses to wild-type, symptoms have been observed in all obligate heterozygotes, with a similar age range for onset to that of homozygotes. We have designated this autosomal dominant disorder Motor neuron degeneration (Mnd).

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Keywords

Animals; Behavior, Animal/physiology; Disease Models, Animal; Female; Genes, Dominant; Male; Mice; Mice, Inbred C57BL; Motor Neurons/pathology; Nerve Degeneration; Neuromuscular Diseases/genetics; Neuromuscular Diseases/pathology; Neuromuscular Diseases/physiopathology; Reproduction

Significance

Annotations

Gene product Qualifier GO ID GO term name Evidence Code with/from Aspect Notes Status


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References

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