Table 1.

Summary of phenotypes of mutant FUS knock-in mouse lines

Genotype	Birth weighta	Suvival	MN loss	Muscle innervation	Other phenotypes
WT/WT	1.40 ± 0.01 g n = 183	Normal	Normal	Normal	NA
P517L/WT	1.37 ± 0.01 g n = 157	~90% survive to weaning	Onset at 1.5 years	TA denervation onset at 1 year	Malocclusion and rectal prolapse (all rare)
Δ14/WT	1.36 ± 0.01 g n = 173	~90% survive to weaning	Onset at 1.5 years	TA denervation onset at 1 year	Malocclusion and rectal prolapse (all rare)
WT/KO	1.34 ± 0.02 g n = 95	Normal	Normal	Normal	No known phenotype
KO/KO	1.00 ± 0.02 g n = 26	Perinatal lethal	Normal	Normal at birth	Hicks et al., 2000
P517L/KO	1.15 ± 0.02 g n = 14	21 ± 12 d	NA	Normal at birth	Malocclusion, rectal prolapse and seizures
Δ14/KO	1.02 ± 0.02 g n = 13	Perinatal lethal; max survival ~1 week	NA	NA	NA
P517L/P517L	0.98 ± 0.02 g n = 26	Perinatal lethal	Normal at birth	Normal at birth	NA
Δ14/Δ14	1.09 ± 0.01 g n = 50	~20% survive to weaning	Onset at 1 year	NA	Malocclusion, rectal prolapse and seizures
P517L/Δ14	1.10 ± 0.02 g n = 44	Perinatal lethal; max survival ~3 weeks	NA	NA	NA
MN-Δ14/WT: c14/WT; ChAT-Cre	NA	NA	Onset at 1.5 years	NA	NA
MN-Δ14/Δ14: c14/c14; ChAT-Cre c14/Δ14; ChAT-Cre	NA	NA	Onset at 1 year	NA	NA
MN-P517L/Δ14: P517L/c14; ChAT-Cre	1.42 ± 0.03 g n = 28	~90% survive to weaning	Onset at 4 months	TA denervation onset at 4 months	Malocclusion, rectal prolapse and seizures (all rare)

^aMean ± s.e.m. n represents number of individual animals. NA, not available.