About   Help   FAQ
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)
• 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 (J:19328)
• focal thinning of the retinal pigment epithelium occurs at late stages of degeneration (J:19328)
• at P15, the outer nuclear layer (ONL) contains greater number of pyknotic nuclei and is thinner (J:19328)
• rapid thinning of the ONL by P25, with a more gradual thinning at later ages (J:19328)
• at P15, the outer nuclear layer (ONL) contains greater number of pyknotic nuclei and is thinner (J:19328)
• rapid thinning of the ONL by P25, with a more gradual thinning at later ages (J:19328)
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages (J:19328)
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages (J:19328)
• at very late stages of degeneration, inner segments are shortened and broadened (J:19328)
• at very late stages of degeneration, inner segments are shortened and broadened (J:19328)
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages (J:19328)
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages (J:19328)
• progressive shortening of the outer segments while maintaining relatively normal structure (J:19328)
• progressive shortening of the outer segments while maintaining relatively normal structure (J:19328)
• detectable at P15 and more pronounced with age (J:19328)
• detectable at P15 and more pronounced with age (J:19328)

pigmentation
• focal thinning of the retinal pigment epithelium occurs at late stages of degeneration (J:19328)
• focal thinning of the retinal pigment epithelium occurs at late stages of degeneration (J:19328)

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 (J:1224)
• 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 (J:1224)
• Background Sensitivity: motor neuron disease is accelerated with 40% exhibiting symptoms by 4.5 months of age and dying by 6.5-7 months (J:56219)
• Background Sensitivity: motor neuron disease is accelerated with 40% exhibiting symptoms by 4.5 months of age and dying by 6.5-7 months (J:56219)
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages (J:19328)
• rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages (J:19328)
• at very late stages of degeneration, inner segments are shortened and broadened (J:19328)
• at very late stages of degeneration, inner segments are shortened and broadened (J:19328)
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages (J:19328)
• rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages (J:19328)
• progressive shortening of the outer segments while maintaining relatively normal structure (J:19328)
• progressive shortening of the outer segments while maintaining relatively normal structure (J:19328)

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
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
01/26/2016
MGI 6.02
The Jackson Laboratory