Table 1. Phenotype summary for hemizygous SOD1^G93A transgenic mice, heterozygous Dync1h1^Loa/+ and Gars^{C201R/ +} mouse strains.

Mouse strain	SOD1G93A	Dync1h1Loa	GarsC201R
Type of mutation	Transgenic overexpressor of human mutant superoxide dismutase 1 gene	ENU induced point mutation in endogenous mouse cytoplasmic dynein heavy chain 1 gene, resulting in missense mutation in protein, phenylalanine to tyrosine at residue 580	ENU induced point mutation in endogenous mouse glycyl-RNA synthetase gene, resulting in missense mutation in protein, cysteine to arginine at residue 201
Primary reference	[19]	[22]	[25]
Genetic data	Autosomal dominant trait; transgene inserted into mouse chromosome 12 [27]	Autosomal dominant mutation on mouse chromosome 12	Autosomal dominant mutation on mouse chromosome 6
Human disease model	Amyotrophic lateral sclerosis	None described	Charcot-Marie-Tooth type 2D (some features)
Breeding	Females are infertile; equal numbers of male and female progeny are produced	Males and females are fully fertile; equal numbers of male and female progeny are produced	Males and females are fully fertile; equal numbers of male and female progeny are produced
Lifespan	∼130 days for humane endpoint, depending on genetic background	Over 2 years	Over 2 years
Age at onset	∼90 days depending on genetic background	1–3 months depending on genetic background	At least 1 month depending on genetic background
Symptoms at onset	Paralysis and weight loss	Limb clasping when suspended by the tail; low based gait in some mice	Mild deficits in grip strength and fine motor control
Nerve and muscle	Motor neuron degeneration, loss of muscle force, muscle atrophy	Mild motor neuron loss; pronounced loss of prioprioceptors, some loss of muscle force, no obvious muscle pathology so far	Reduction in axon diameter of peripheral nerves, alteration in sensory nerve conduction, neuromuscular junction deficits; some loss of muscle force, changes in muscle fiber type
Mouse crosses	Crossed to Dync1h1Loa [26]; crossed to GarsC201R this paper	Crossed to GarsC201R this paper; crossed to SOD1G93A [26]	Crossed to SOD1G93A this paper; crossed to Dync1h1Loa this paper

Note that the phenotype of homozygous Dync1h1^Loa/+ and Gars^{C201R/ +} mice is considerably more severe and few homozygous animals of either strain survive much beyond birth. Mice with two copies of the SOD1^G93A transgene array are not viable.