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

• 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

Mouse Models of Human Disease		OMIM ID	Ref(s)
Amyotrophic Lateral Sclerosis 10, with or without Frontotemporal Dementia;		612069	J:165985