Phenotypes Associated with This Genotype

Genotype
MGI:4438494

• Allelic Composition: Tg(Thy1-TARDBP)4Singh/0
• Genetic Background: involves: C57BL/6J * SJL/J

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

behavior/neurological

limb grasping ( J:157550 )

• abnormal limb reflex at about 14 months of age

impaired coordination ( J:157550 )

• 40% reduced motor performance at about 15 months of age

nervous system

microgliosis ( J:157550 )

• highly transgene dose dependent

abnormal hippocampus morphology ( J:157550 )

• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons

abnormal subiculum morphology ( J:157550 )

• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons

abnormal neocortex morphology ( J:157550 )

• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex

abnormal somatosensory cortex morphology ( J:157550 )

• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs

Purkinje cell degeneration ( J:157550 )

astrocytosis ( J:157550 )

• highly transgene dose dependent

neuronal intranuclear inclusions ( J:157550 )

• present in cortical layer V of the anterior cortex including the primary motor cortex and somatosensory areas of the hind- and forelimbs and to some extent in the hippocampal/subicular neurons

abnormal spinal cord ventral horn morphology ( J:157550 )

• atrophy and increased number of pyknotic neurons in the ventral horn region of the lumbosacral and cervical spinal cord occurs in a transgene dose dependent manner

hematopoietic system

microgliosis ( J:157550 )

• highly transgene dose dependent

immune system

microgliosis ( J:157550 )

• highly transgene dose dependent

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 10		DOID:0060201	OMIM:612069	J:157550
frontotemporal dementia		DOID:9255	OMIM:600274	J:157550