Mouse Genome Informatics
cx
    Tg(Camk2a-tTA)1Mmay/0
Tg(tetO-HTT*94Q,-lacZ)1Rhn/0

involves: C57BL/6 * CBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• some mice die between 8 to 10 months due to injuries sustain from fighting with other mice
• fewer than expected mice are recovered due to prenatal lethality
• however, treatment of pregnant dams with doxycycline eliminates prenatal lethality

nervous system
• ventricles are enlarged compared to in wild-type mice
• the striatum is reduced in size compared to in wild-type mice
• the ventricular zone of the striatum is narrower than in wild-type mice
• the area of the caudate putamen is reduced compared to in wild-type mice
• however, treatment with doxycycline after the onset of symptoms restores some of the caudate putamen area
• gliosis spreads throughout the lateral and medial striatum over time
• mice exhibit reactive astrocytosis in the striatum that spreads over time
• however, treatment with doxycycline after the onset of symptoms reduces astrocytosis
• mice develop neuronal intranuclear and extranuclear inclusion
• however, treatment with doxycycline after the onset of symptoms alleviates inclusions

behavior/neurological
• in older mice with tremors
• beginning at 4 weeks, some mice exhibit limb grasping when suspended
• by 8 weeks, all mice display clasping that can last after mice are released
• however, treatment with doxycycline after the onset of symptoms reduces glasping behavior and duration
• mild tremors begin at 20 weeks in some mice
• in some mice tremors develop into a jerking motion
• mice exhibit progressive motor dysfunction with choreic and dystonic movements, and parkinsonism
• at 36 weeks

muscle
• mice exhibit progressive motor dysfunction with choreic and dystonic movements, and parkinsonism

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:61490