Mice hemizygous for this SOD1-G93A (also called G93A-SOD1) transgene are viable and fertile, with transgenic expression of a G93A mutant form of human SOD1. This founder line (often referred to as G1H) is reported to have high transgene copy number. Hemizygotes exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans; becoming paralyzed in one or more limbs with paralysis due to loss of motor neurons from the spinal cord. Motor neuron degeneration has been associated with function and/or degeneration of astrocytes, the major glial cell type of the nervous system. Transgenic mice have an abbreviated life span: 50% survive at 157.1+/-9.3 days (in contrast to the mixed B6SJL background where 50% survival is observed at 128.9+/-9.1 days). Female hemizygotes are poor breeders, and rarely produce more than one litter before the onset of disease. In contrast to LPS-induced microglia and activated M1/M2 macrophages, spinal cord microglia activated by disease progression do not upregulate genes that display a bias to either an M1 (neurotoxic) phenotype or an M2 (protective) phenotype. The pattern of gene expression in SOD1G93A activated microglia represents a unique ALS-specific signature. These SOD1-G93A (also called G93A-SOD1) transgenic mice may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease). 
 This strain ships with remnant UID MiniMax microchips implanted subcutaneously in the back. For more information on these RFID chips, including our FAQ, please visit our <a href="https://www.jax.org/jax-mice-and-services/customer-support/technical-support/breeding-and-husbandry-support/mouse-identification">Mouse Identification page</a>.
 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. As the SOD1-G93A transgenic mice were originally created on a mixed genetic background, it should be noted that the phenotype of the congenic mice (Stock No. 004435) could vary from that originally described. We will modify the strain description if necessary as published results become available.

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This strain ships with remnant UID MiniMax microchips implanted 
subcutaneously in the back. For more information on these RFID chips, 
including our FAQ, please visit our <a href="https://www.jax.org/jax-mice-and-services/customer-support/technical-support/breeding-and-husbandry-support/mouse-identification">Mouse Identification page</a>. SOD1-G93A transgenic mice express a G93A mutant form of human SOD1 and may be useful in studying neuromuscular disorders such as Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease). Our preclinical efficacy testing services offer scientific expertise and an array of target-based and phenotype-based outcome measures, both in vivo and at endpoint, for flexible study designs and assay development in mouse models of Amyotrophic Lateral Sclerosis. <a href="https://www.jax.org/jax-mice-and-services/in-vivo-pharmacology/neurobiology-services/amyotrophic-lateral-sclerosis-efficacy-studies">See our full service platform</a>. This SOD1-G93A transgene is available on C57BL/6J (Stock No. 004435), B6SJL (Stock No. <a href="http://www.jax.org/strain/002726">002726</a>) and FVB/NJ (Stock No. <a href="http://www.jax.org/strain/013199">013199</a>). In addition, a very low expressing variant on B6SJL is available (Stock No. <a href="http://www.jax.org/strain/032166">032166</a>).

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