Phenotypes Associated with This Genotype

Genotype
MGI:6101467

• Allelic Composition: Fus^tm1.1Emcf/Fus⁺
• Genetic Background: involves: C3H * C57BL/6J * C57BL/6N

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

mortality/aging

premature death ( J:249965 )

• modest reduction in survival at 22 months

nervous system

decreased motor neuron number ( J:249965 )

• at 12 and 18 months, but not 3 months

motor neuron degeneration ( J:249965 )

• in aging mice without protein aggregates

• reduced motor units in the extensor digitorum at 18 months

• reduced number of intact neuromuscular junctions at 18 months in hindlimb lumbrical muscles

• however, motor neurons are normal at 3 months

abnormal neuromuscular synapse morphology ( J:249965 )

• reduced number of intact neuromuscular junctions at 18 months in hindlimb lumbrical muscles

behavior/neurological

impaired limb coordination ( J:249965 )

• at 12 and 15 months, mice exhibit hindlimb locotronic errors

abnormal gait ( J:249965 )

• altered rear stride at 18 months with reduction in the time the rear limb is in the swing phase

• however, stride length is normal

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 6		DOID:0060198	OMIM:608030	J:249965