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Phenotypes Associated with This Genotype
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cln8mnd mutation (2 available); any Cln8 mutation (9 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• Background Sensitivity: most die by 9-14 months of age (J:8492)

• hindlimbs are unable to grasp the bars of wire cagetop when attempting to walk over it
• unable to walk uphill
• dragging or splaying of the hindlimbs while walking
• age of onset is approximately 5- 11 months of age, progressive with age
• progress to severe spastic paresis and paralysis by 9 months of age
• age of onset is approximately 5- 11 months of age, progressive with age
• begin to develop paresis by 6 months of age

nervous system
• contain LFB-positive intracytoplasmic inclusion material in most neurons in virtually all parts of the brain and spinal cord unlike in controls
• inclusion bodies containing ubiquitin were found in spinal neurons of mnd mice, even prior to onset of symptoms (J:8492)
• motor neurons have eccentric or indiscrete nuclei, disrupted membranes and shape changes (J:8492)
• Background Sensitivity: exhibit motor neuron disease symptoms at around 6 months of age (J:56219)
• degeneration of the upper and lower motor neurons of the spinal cord and cranial nerves and of some areas of the brain
• degeneration of cranial nerves
• degenerating dorsal motor vagus
• degeneration of anterior horn cells in the spinal cord

reproductive system
• lower number of progeny/litter and lower numbers of total litters

• atrophy of the photoreceptor layer in mice older than 3 months and nearly complete loss of this layer by 8 months of age
• begin to become blind by 2 months of age and by 5 months, are completely blind

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
neuronal ceroid lipofuscinosis 8 DOID:0110723 OMIM:600143
J:12816 , 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
MGI 6.10
The Jackson Laboratory