All Phenotypes Fus<tm1Ics> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Fus^tm1Ics/Fus⁺	involves: C57BL/6	MGI:6716017
cn2	Chat^tm1(cre)Lowl/Chat⁺ Fus^tm1Ics/Fus⁺	involves: 129S6/SvEvTac * C57BL/6	MGI:8435668

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

behavior/neurological

abnormal gait ( J:371167 )

• mice show irregular walking pattern characterized by phases of fast walking interrupted with stance phases

• mice show impaired swing and body speed at 22 months of age

short stride length ( J:371167 )

• reduction in hind limb stride length associated with an increase in body speed variation at 10 and 22 months of age

cellular

increased oligodendrocyte number ( J:371167 )

• oligodendrocytes are more numerous in ventral horn white matter in 22-month-old mice

muscle

abnormal muscle electrophysiology ( J:371167 )

• 22-month-old mice show typical fibrillation and fasciculation potentials in gastrocnemius and tibialis anterior muscle indicating the presence of spontaneous denervation activities

• electromyography shows increased frequency of abnormal potentials in gastrocnemius and tibialis anterior muscle at 22, but not 10, months of age and the amplitude of compound muscle action potentials is decreased in 18- to 22- month-old mice

muscle weakness ( J:371167 )

• mice show shorter hanging time and decreased holding impulse (represents the total sustained force exerted by the mouse to oppose the gravitational force) in an inverted grid test at 10 and 22 months of age

• however, mice show normal grip strength in the grip test and performance on the rotarod and do not develop paralysis

nervous system

increased oligodendrocyte number ( J:371167 )

• oligodendrocytes are more numerous in ventral horn white matter in 22-month-old mice

abnormal motor neuron morphology ( J:371167 )

• motor neurons exhibit cytoplasmic accumulation of methylated FUS and ubiquitin pathology but no large FUS aggregates

• motor neuron axons in ventral roots show a distribution shifted towards smaller caliber in 22-month-old mice

motor neuron degeneration ( J:371167 )

• degeneration of motor neurons in the lumbar spinal cord is seen at 22 months, but not 10 months of age, with an approximate 30% reduction in number of motor neurons, indicating progressive degeneration

abnormal myelination ( J:371167 )

• mice at 22 months of age show a defect in myelination of motor axons, with a modest decrease in g-ratio for ventral root axons of smaller calibers

• fewer axons show normal myelination in ventral roots

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 6		DOID:0060198	OMIM:608030	J:371167

Allelic Composition	Chat^tm1(cre)Lowl/Chat⁺ Fus^tm1Ics/Fus⁺
Genetic Background	involves: 129S6/SvEvTac * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Chat^tm1(cre)Lowl mutation (1 available); any Chat mutation (63 available)
Fus^tm1Ics mutation (0 available); any Fus mutation (59 available)

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

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:371167 )

• at 22 months of age, but not 10 months of age, mice show impaired motor performance, indicating delayed motor deficits

cellular

increased oligodendrocyte number ( J:371167 )

• oligodendrocytes are more numerous in ventral horn white matter in 22-month-old mice

muscle

muscle weakness ( J:371167 )

• 22-month-old mice show shorter hanging time and decreased holding impulse (represents the total sustained force exerted by the mouse to oppose the gravitational force) in an inverted grid test

nervous system

nervous system phenotype ( J:371167 )

N	• mice show prevention of motor neuron degeneration, with normal motor neuron numbers at 22 months of age and do not develop electromyography abnormalities

increased oligodendrocyte number ( J:371167 )

• oligodendrocytes are more numerous in ventral horn white matter in 22-month-old mice

abnormal motor neuron morphology ( J:371167 )

• motor neuron axons show a distribution shifted towards smaller caliber in 22-month-old mice

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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