Anti-Superoxide Dismutase, Cu,Zn (Superoxide Dismutase Cu/Zn, SOD Cu,Zn, Cu/Zn SOD, Amyotrophic Late

Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Catalytic activity: 2 superoxide + 2 H+ = O2 + H2O2. COFACTOR: Binds 1 copper ion per subunit. Cofactor: Binds 1 zinc ion per subunit. Subunit: Homodimer. , Zinc binding promotes dimerization. Belongs to the Cu-Zn superoxide dismutase family. Defects in SOD1 are the cause of familial amyotrophic lateral sclerosis (FALS), also called amyotrophic lateral sclerosis 1 (ALS1 or ALS). ALS is a degenerative disorder of motorneurons in the cortex, brainstem and spinal cord. ALS is characterized by muscular weakness and atrophy beginning in the hands and spreading to the forearms and legs. Muscle fasciculations are commonly visible. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. ALS is sometimes referred to as Lou Gehrig disease after the famous American baseball player who was diagnosed with the disorder. FALS, the familial form of ALS, accounts for about 10% of the cases and is transmitted in an autosomal dominant manner. The mean age at onset of FALS is 45 years. , Applications: , Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Cellular Localization: Cytoplasm, Storage and Stability: May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.