Phenotypes Associated with This Genotype

Genotype
MGI:5448852

• Allelic Composition: Tg(Camk2a-tTA)1Mmay/0; Tg(tetO-TARDBP)12Vle/0
• Genetic Background: involves: C3H/HeJ * C57BL/6J

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

nervous system

microgliosis ( J:170756 )

• microglial activation is seen in the cortex and hippocampus as early as 1 month after Dox removal

decreased brain weight ( J:170756 )

• 6 months following Dox removal

abnormal dentate gyrus morphology ( J:170756 )

• 20% loss of dentate gyrus neurons at 1 month and about 75% loss by 3 months after Dox removal

astrocytosis ( J:170756 )

• astrocytic activation is seen in the cortex and hippocampus as early as 1 month after Dox removal

neurodegeneration ( J:170756 )

• mutants switched to a doxycycline (Dox)-free diet at 28 days of age show progressive neurodegeneration, with neuron loss in the hippocampal dentate gyrus and neocortex beginning at about 1 month after Dox removal

behavior/neurological

limb grasping ( J:170756 )

• variable clasping behavior is seen approximately 1-3 months after Dox removal

hematopoietic system

microgliosis ( J:170756 )

• microglial activation is seen in the cortex and hippocampus as early as 1 month after Dox removal

immune system

microgliosis ( J:170756 )

• microglial activation is seen in the cortex and hippocampus as early as 1 month after Dox removal

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 10		DOID:0060201	OMIM:612069	J:170756