Target Misfolded SOD1 Target background Superoxide dismutase 1 (SOD1) is a soluble cytoplasmic and mitochondrial intermembrane space protein. SOD1 binds copper and zinc ions and is one of three isozymes responsible for destroying free superoxide radicals in the body. Mutations in SOD1 cause familial amyotrophic lateral sclerosis type 1 (ALS1). These mutations have been linked to accumulation of harmful superoxide radicals, promotion of apoptosis, formation of aggregates of misfolded superoxide dismutase which are toxic and the continued stimulation of nerve cells that causes them to burn out and die. Target alias Superoxide dismutase [Cu-Zn], Superoxide dismutase 1, hSOD1, SOD1 Immunogen Recombinant human SOD1 G93A APO Specificity The antibody recognizes misfolded forms of mutant human SOD1 protein. By native blot, C4F6 is specific for misfolded forms of SOD1 in G93A mutant model, and oxydized SOD1 models compared to normal SOD1. By IF, C4F6 is specific for misfolded SOD1 from G93A mutant model vs. wt SOD1. C4F6 clone is also able to recognize different conformers of misfolded SOD1 under specific conditions detailed in various publications. Clone ID C4F6 Isotype IgG2a Preservative None Format Purified with protein G, stored in PBS pH 7.4 and lyophilized. Recommend starting dilution Each tube contains 50 µg of purified antibodies. If reconstituted with deionized water in 200 µL: IF 1:50 to 1:500; WB 1:250; IP 6 µL / 40 µL (Protein G beads / 300 µg of protein lysate). Optimal dilution has to be determined by the user. Limitations Research Use Only Storage Lyophilized antibodies can be kept at 4ºC for up to 3 months and should be kept at -20ºC for long-term storage (2 years). To avoid freeze-thaw cycles, reconstituted antibodies should be aliquoted before freezing for long-term (1 year) storage (-80ºC) or kept at 4ºC for short-term usage (2 months). For maximum recovery of product, centrifuge the original vial prior to removing the cap. Further dilutions can be made with the assay buffer. After the maximum long-term storage period (2 years lyophilized or 1 year reconstituted) antibodies should be tested in your assay with a standard sample to verify if you have noticed any decrease in their efficacy. To limit antibody loss or degradation, BSA (final concentration 1%) and sodium azide (final concentration 0.02%) can be added to the suggested first dilution. It is important to first verify if those preservatives are compatible with your assay.