Phenotypes Associated with This Genotype

Genotype
MGI:6473661

• Allelic Composition: Gt(ROSA)26Sor^{tm1(CAG-SNCA)Tanj}/Gt(ROSA)26Sor^{tm1(CAG-SNCA)Tanj}; Tg(Plp1-cre/ERT)3Pop/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6J * C57BL/6NCrl * DBA/2

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

mortality/aging

premature death ( J:283567 )

♂ • approximately 30% of males exhibit sudden death 20 weeks after tamoxifen treatment

behavior/neurological

impaired coordination ( J:283567 )

• mice show deficits in motor coordination and balance on the rotarod at 20 to 50 weeks after tamoxifen induction

• however, tamoxifen induced mice show no differences in immobility in the forced swim test from controls

decreased locomotor activity ( J:283567 )

• 20 to 50 weeks after tamoxifen induction, mice show deterioration of performance in the open field, having shorter moving distance

• however, tamoxifen treated mice do not show muscle abnormalities and no differences in grip handling

nervous system

nervous system phenotype ( J:283567 )

N • tamoxifen treated mice do not show neuronal loss in the anterior horn, the cerebellum, brainstem, substantia nigra and striatum

abnormal glial cell morphology ( J:283567 )

• ubiquitin accumulation is seen in glial cells after tamoxifen induction

• tamoxifen-treated mice show increased GFAP+ astrocytes and Iba1+ microglial cells in the brain stem indicating accelerated inflammatory response with age

abnormal neuron morphology ( J:283567 )

• ubiquitin accumulation is seen in neurons after tamoxifen induction

alpha-synuclein inclusion body ( J:283567 )

• tamoxifen treated mice show alpha-synuclein inclusions mainly aggregated in oligodendrocytes

• perinuclear alpha-synuclein inclusions are seen in certain neurons, especially those located in the brainstem and putamen, 1 week after tamoxifen induction

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
multiple system atrophy		DOID:4752		J:283567