Phenotypes associated with this allele

• Allele Symbol: Tg(Prnp-TARDBP*A315T)23Jlel
• Allele Name: transgene insertion 23, Jeffrey L Elliott
• Allele ID: MGI:4839643
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Prnp-TARDBP*A315T)23Jlel/0	involves: C57BL/6 * CD-1 * SJL	MGI:4839653

Genotype
MGI:4839653

tg1

• Allelic Composition: Tg(Prnp-TARDBP*A315T)23Jlel/0
• Genetic Background: involves: C57BL/6 * CD-1 * SJL

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

mortality/aging

premature death ( J:165985 )

• mean survival is 75 days

nervous system

abnormal brainstem morphology ( J:165985 )

• brainstem neurons exhibit ubiquitin inclusions and astrogliosis unlike in wild-type mice

astrocytosis ( J:165985 )

• in the ventral horn and brainstem neurons

neuronal intranuclear inclusions ( J:165985 )

• rare with TARDBP co-localization

abnormal spinal cord ventral horn morphology ( J:165985 )

• mice exhibit marked astrogliosis compared with wild-type mice

• ventral horn neurons exhibit prominent ubiquitin positive cytoplasmic inclusions unlike in wild-type mice

• however, no pale amorphous cyto-architectural abnormalities observed in early onset severe founders that fail to exhibit germline transmission

behavior/neurological

abnormal motor coordination/balance ( J:165985 )

• mice exhibit late onset, progressive motor defects compared with wild-type mice

decreased grip strength ( J:165985 )

• progressive

short stride length ( J:165985 )

• progressive

muscle

abnormal quadriceps morphology ( J:165985 )

• quadriceps muscle exhibits atrophy and angular fibers consistent with denervation changes compared with wild-type mice

skeletal muscle atrophy ( J:165985 )

• in the quadriceps muscle

muscle weakness ( J:165985 )

• in the hindlimb

limbs/digits/tail

abnormal quadriceps morphology ( J:165985 )

• quadriceps muscle exhibits atrophy and angular fibers consistent with denervation changes compared with wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 10		DOID:0060201	OMIM:612069	J:165985