84-1131-41　［取扱停止］Superoxide Dismutase, Cu,Zn (Superoxide Dismutase Cu/Zn, SOD Cu,Zn, Cu/Zn SOD, CuZnSOD, SODC, Amyotrophic Lateral Sclerosis 1, ALS1, ALS, Ipo1, Ipo-1, Superoxide Dismutase 1 (Soluble), SOD1, SOD-1) 100ul　S8060-01M

特徴

• FUNCTION: 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.
• SUBCELLULAR LOCATION: Cytoplasm.
• DISEASE: 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.
• MISCELLANEOUS: Zinc binding promotes dimerization.
• SIMILARITY: Belongs to the Cu-Zn superoxide dismutase family.
• Applications: Suitable for use in Western Blot.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:1000Optimal dilutions to be determined by the researcher.
• Storage and Stability:Lyophilized powder may be stored at -20°C.
• Stable for 12 months at -20°C.
• Reconstitute with sterile ddH2O.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Reconstituted product is stable for 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.