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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.
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.
