These mice carry a spontaneous mutation at the Cln8 locus characterized by hindlimb weakness and ataxia. They are suitable for use in applications related to the study of neurodegenerative disease.Read More +
Cln8mnd mice are 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.
|Allele Name||motor neuron degeneration|
|Allele Synonym(s)||Cln8mnd; mnd|
|Gene Symbol and Name||Cln8, ceroid-lipofuscinosis, neuronal 8|
|Strain of Origin||B6.KB2-H2b5|
|General Note|| |
Early papers (J:8492, J:1224) state that this allele exhibits phenotypic similarity to amytrophic lateral sclerosis (ALS), however further analysis (J:12816, 56219) revealed that it is a better model for neuronal ceroid lipofuscinoses (Batten's disease) than for ALS.
|Molecular Note||A single nucleotide insertion (267-268C, codon 90) predicts a frameshift and a truncated protein.|
When using the motor neuron degeneration mouse strain in a publication, please cite the originating article(s) and include JAX stock #003906 in your Materials and Methods section.