Mouse Genome Informatics
involves: 129S1/Sv * C57BL/6
phenotype observed in females
phenotype observed in males
N normal phenotype
• behavioral symptoms preceed neuropathology which becomes intense and widespread at 4 months of age
• males do not display the high level of climbing activity shown by control males
• increase in rearing at 1 month of age
• increase in locomotor activity and rearing at 1 month of age (J:85298)
• females do more dark phase running than controls (J:141673)
• the observed hyperactivity is followed by hypoactivity at 4 months of age (J:85298)
• less time spent on a running wheel during the light phase (J:141673)
• gait abnormalities develop by 1 year of age, with smaller stride lengths
• develops by 1 year of age

nervous system
• exhibit neuropathological aggregates of mutant protein in various regions of the brain that appear several months after the onset of behavioral symptoms
• regions in which nuclear and neuropil aggregates are found include the striatum, cerebral cortex, hippocampus, pallidum, globus pallidus, and amygdala

• exhibit the progressive appearance of neuropathological mutant protein aggregates in the olfactory tubercle, olfactory bulb, anterior olfactory nucleus, piriform cortex, and entorhinal cortex

• female mice weigh less than controls

Mouse Models of Human Disease
Huntington Disease; HD 143100 J:85298 , J:141673