These Thy1.2-G93A transgenic mice have a human SOD1 cDNA harboring the G93A mutation driven by the murine Thy1.2 expression cassette. Expression of the G93A mutant SOD1 (G93A-SOD1) is directed to neurons throughout the brain and spinal cord (including spinal motor neurons). In addition, mutant SOD1 immunoreactivity is selectively distributed in axons and nerve endings at the neuromuscular junctions. While Thy1.2-G93A hemizygous mice from founder line T3 are viable and fertile with no clinical or pathological signs of motor abnormalities up to two years of age, neuronal expression of mutant SOD1 in homozygous Thy1.2-G93A mice from founder line T3 (also called T3T3 mice) develop an Amyotrophic Lateral Sclerosis (ALS)-like motor neuron disease between one to two years of age, characterized by motor neuron degeneration, muscle denervation, paralysis, and muscle weakness with cytosolic dendritic ubiquitinated SOD1 aggregates as the dominant pathological feature. Additionally, when these T3 mice are bred with N29 hSOD1 mice (see Tg(SOD1)2Gur mice; Stock No. 002297 and 002298), T3/hSOD1 double hemizygous offspring develop an ALS-like motor neuron disease between one to two years of age. These Thy1.2-G93A (or T3) transgenic mice may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).
In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype of these mice could vary from that originally described. We will modify the strain description if necessary as published results become available.
