About   Help   FAQ
Cln8mnd
Spontaneous Allele Detail
Nomenclature
Symbol: Cln8mnd
Name: ceroid-lipofuscinosis, neuronal 8; motor neuron degeneration
MGI ID: MGI:1856959
Synonyms: Cln8mnd, mnd
Gene: Cln8  Location: Chr8:14881335-14901720 bp, + strand  Genetic Position: Chr8, 7.59 cM
Mutation
origin
Strain of Origin:  B6.KB2-H2b5
Mutation
description
Allele Type:    Spontaneous
Mutation:    Insertion
    A single nucleotide insertion (267-268C, codon 90) predicts a frameshift and a truncated protein. (J:57766)
Inheritance:    Recessive
Phenotypes
Loading...
View phenotypes for all genotypes (concatenated display).
Disease models
Loading...
Find Mice (IMSR)
Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 2 strains available      Cell Lines: 0 lines available
Carrying any Cln8 Mutation:  6 strains or lines available
Notes

It was originally thought that heterozygotes (mnd/+) could be detected at very old ages. However, it has been impossible to repeat these results and, even in 18-month old heterozygotes, no histological pathology is detectable (A. Messer and R. Bronson, personal communication, 1993).

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.

References
Original:  J:8492 Messer A, et al., Autosomal dominance in a late-onset motor neuron disease in the mouse. J Neurogenet. 1986 Nov;3(6):345-55
All:  50 reference(s)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
10/21/2014
MGI 5.20
The Jackson Laboratory