Phenotypes Associated with This Genotype

Genotype
MGI:5052381

• Allelic Composition: Tg(HTT*/EGFP)150Nn/0
• Genetic Background: involves: C57BL/6 * DBA/2

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:174269 )

• median survival is 32 weeks

• all die by 58 weeks of age

nervous system

abnormal nervous system morphology ( J:174269 )

• progression of neuropathology is less rapid compared to mice carrying Tg(HTT*/EGFP)190Nn

abnormal striatum morphology ( J:174269 )

• dense EGFP positive aggregates are detected mostly in the cytoplasm at 35 weeks of age

abnormal hippocampus morphology ( J:174269 )

• dense EGFP positive aggregates are detected mostly in the cytoplasm at 35 weeks of age

abnormal cerebral cortex morphology ( J:174269 )

• dense EGFP positive aggregates are detected mostly in the cytoplasm at 35 weeks of age

neuronal intranuclear inclusions ( J:174269 )

• a few dense EGFP positive aggregates are detected in the nucleus but most are localized to the cytoplasm in the striatum, cortex and hippocampus at 35 weeks of age

• aggregates appear in both the nucleus and cytoplasm at 4 weeks of age but nuclear aggregates gradually lose EGFP fluorescence with age

behavior/neurological

abnormal involuntary movement ( J:174269 )

• chorea-like movements

• show irregular, jerky involuntary head movements at about 16 weeks of age

limb grasping ( J:174269 )

• rare

tremors ( J:174269 )

• fine tremor with onset at about 9-11 weeks of age

ataxia ( J:174269 )

• progressive development of an ataxic gait that is severe and interferes with walking by 30 weeks of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:174269