Mouse Genome Informatics
hm
    Cln8mnd/Cln8mnd
B6.KB2/Rn-Cln8mnd
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• Background Sensitivity: most die by 9-14 months of age (J:8492)

behavior/neurological
• 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

vision/eye
• 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
OMIM IDRef(s)
Ceroid Lipofuscinosis, Neuronal, 8; CLN8 600143 J:12816 , J:56219