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Phenotypes Associated with This Genotype
Genotype
MGI:2388401
Allelic
Composition
Cln8mnd/Cln8mnd
Genetic
Background
involves: AKR/J * B6.KB2
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cln8mnd mutation (2 available); any Cln8 mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: death by 7 months of age, compared to 9-14 months on the inbred B6.KB2 background (J:1224)

vision/eye
• focal thinning of the retinal pigment epithelium occurs at late stages of degeneration
• at P15, the outer nuclear layer (ONL) contains greater number of pyknotic nuclei and is thinner
• rapid thinning of the ONL by P25, with a more gradual thinning at later ages
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages
• at very late stages of degeneration, inner segments are shortened and broadened
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages
• progressive shortening of the outer segments while maintaining relatively normal structure
• detectable at P15 and more pronounced with age

pigmentation
• focal thinning of the retinal pigment epithelium occurs at late stages of degeneration

nervous system
• Background Sensitivity: earlier age of onset (4.5-5 months) and increased speed of progression of neurological disease than on the inbred B6.KB2 background
• Background Sensitivity: motor neuron disease is accelerated with 40% exhibiting symptoms by 4.5 months of age and dying by 6.5-7 months
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages
• at very late stages of degeneration, inner segments are shortened and broadened
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages
• progressive shortening of the outer segments while maintaining relatively normal structure

Mouse Models of Human Disease
OMIM ID Ref(s)
Ceroid Lipofuscinosis, Neuronal, 8; CLN8 600143 J:56219


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
09/13/2016
MGI 6.05
The Jackson Laboratory