Mouse Genome Informatics
involves: C57BL/6 * CBA
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• reduction in brain weight by 5 weeks
• immunoreactive htt is detected in the striatum at 4.5 weeks
• immunoreactive htt is detected in the cortex beginning at 3.5 weeks
• striatal neurons have prominent and frequent indentations of the nuclear membrane and an apparent increase in the clustering and number of nuclear pores by 10-12 weeks of age
• inclusions are observed within neurons of cerebral cortex, striatum, cerebellum, spinal cord and to a much lesser degree in the hippocampus, thalamus, globus pallidus and substantia nigra
• inclusions appear in the cerebral cortex before they can be detected in the striatum
• inclusions are ubiquitinated by 5-6 weeks and can be detected by ultrastructural analysis by 8 weeks
• htt immunoreactive inclusions are seen in approximately 20% of neurons
• in the striatum, ultrastructural analysis of inclusions reveals a prominent, roughly circular, pale structure
• inclusions are granular with occasional filamentous structures around the periphery; they are larger than the nucleolus and occupy 1% of nuclear volume

• progressive loss of body weight

Mouse Models of Human Disease
Huntington Disease; HD 143100 J:42085