AK.B6-Cln8^mnd/J

Stock number: 003906
Product name: motor neuron degeneration
Research areas: Neurobiology Research, Sensorineural Research

Description

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.

Detailed description

Cln8^mnd 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

Symbol: Cln8^mnd
Name: motor neuron degeneration
MGI accession ID: MGI:1856959
Generation method: Spontaneous
Attributes: Null/Knockout
Marker symbol: Cln8
Marker name: ceroid-lipofuscinosis, neuronal 8
Chromosome: 8
Strain of origin: B6.KB2-H2^b5
Molecular note: A single nucleotide insertion (267-268C, codon 90) predicts a frameshift and a truncated protein.
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.