In homozygous mnd mice, the retinal degeneration occurs before neuromuscular dysfunction. The retinal degeneration was nearly complete by 6 months of age, but the motor neuron abnormalities began around 6 months of age. It is a good model for human Batten diseases, and its expression is clearly recessive.
Gene | Mouse Chromosome | Human Chromosome | Retinal ONL disappear by | Strains |
|---|---|---|---|---|
mnd | 8 | 8p or 13q | 6 | B6.KB2- mnd |