Phenotypes associated with this allele

• Allele Symbol: Tg(Thy1-TARDBP*)BOddo
• Allele Name: transgene insertion B, Salvatore Oddo
• Allele ID: MGI:5310969
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Thy1-TARDBP*)BOddo/Tg(Thy1-TARDBP*)BOddo	C57BL/6-Tg(Thy1-TARDBP*)BOddo	MGI:5689841
tg2	Tg(Thy1-TARDBP*)BOddo/0	C57BL/6-Tg(Thy1-TARDBP*)BOddo	MGI:5310970

Genotype
MGI:5689841

tg1

• Allelic Composition: Tg(Thy1-TARDBP*)BOddo/Tg(Thy1-TARDBP*)BOddo
• Genetic Background: C57BL/6-Tg(Thy1-TARDBP*)BOddo

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

behavior/neurological

impaired spatial learning ( J:223932 )

• 15 month old mice perform worse than wild-type mice on the Morris water maze on day 2-5, traveling longer distances to find the hidden platform, showing fewer platform location crosses and spending less time in the target quadrant

abnormal long-term spatial reference memory ( J:223932 )

• 15 month old homozygotes perform worse than wild-type or hemizygotes in the radial arm water maze showing more entries in arms without platform on day 2, indicating impaired spatial memory

abnormal spatial working memory ( J:223932 )

• 15 month old mutants show fewer number of re-entries in the same arm of the radial arm water maze during each single trial, indicating impaired working memory

impaired coordination ( J:223932 )

• 15 month old homozygotes perform worse than wild-type controls or hemizygotes on the rotarod and on the accelerated rotarod

cellular

abnormal autophagy ( J:223932 )

• marker analysis indicates that autophagy induction is reduced in 15 month old mutants

homeostasis/metabolism

abnormal autophagy ( J:223932 )

• marker analysis indicates that autophagy induction is reduced in 15 month old mutants

abnormal enzyme/coenzyme activity ( J:223932 )

• 15 month old homozygotes show a decrease in chymotrypsin-like, trypsin-like, and caspase-like activities of the proteasome in the brain, indicating a decrease in proteasome function

nervous system

abnormal brain morphology ( J:223932 )

• mice show increased soluble levels of the 25 kDa C-terminal fragment of TARDBP in the nucleus and cytosol

• mice show rare cytosolic TARDBP (TDP-43) deposits

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 10		DOID:0060201	OMIM:612069	J:223932
Grn-related frontotemporal lobar degeneration with Tdp43 inclusions		DOID:0060672	OMIM:607485	J:223932

Genotype
MGI:5310970

tg2

• Allelic Composition: Tg(Thy1-TARDBP*)BOddo/0
• Genetic Background: C57BL/6-Tg(Thy1-TARDBP*)BOddo

behavior/neurological

behavior/neurological phenotype ( J:180165 )

N • 6 month old mutants do not exhibit motor dysfunction, and show normal spontaneous activity, open-field thigmotaxis, time spent in the center of the arena, and rotarod performance

impaired spatial learning ( J:223932 )

• 15 month old mice perform worse than wild-type mice on the Morris water maze on day 2-4, traveling longer distances to find the hidden platform and spending less time in the target quadrant

abnormal long-term spatial reference memory ( J:223932 )

• 15 month old mutants perform worse than controls in the radial arm water maze showing more entries in arms without platform on day 2, indicating impaired spatial memory

abnormal spatial working memory ( J:180165 )

• mutants exhibit impaired performance on the T-maze at 6 months, but not 2 months of age, indicating impaired working memory

abnormal response to novel object ( J:180165 )

• in the novel object recognition task, mutants at 6 months of age, did not discriminate between the novel and old object as in controls, indicating impaired cortical and hippocampal function

impaired coordination ( J:223932 )

• 15 month old mice perform worse than wild-type controls on the rotard and the accelerated rotarod

cellular

abnormal autophagy ( J:223932 )

• marker analysis indicates that autophagy induction is reduced in 15 month old mice

homeostasis/metabolism

abnormal autophagy ( J:223932 )

• marker analysis indicates that autophagy induction is reduced in 15 month old mice

abnormal enzyme/coenzyme activity ( J:223932 )

• 15 month old mice show a decrease in chymotrypsin-like, trypsin-like, and caspase-like activities of the proteasome in the brain, indicating a decrease in proteasome function

nervous system

abnormal brain morphology ( J:223932 )

• 15 month old mice mice show increased soluble levels of the 25 kDa C-terminal fragment of TARDBP in the nucleus and cytosol

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Grn-related frontotemporal lobar degeneration with Tdp43 inclusions		DOID:0060672	OMIM:607485	J:180165