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.
Host
Rabbit
Immunogen
A synthetic peptide (ASGEPVV LSGQIT) as part of mouse superoxide dismutase (SOD1) protein (aa: 24-36) conjugated to diphtheria toxoid
Quantity
100 µl
Reactivity
Rat, Mouse
Recombinant
FALSE
Regulatory
RUO
Shipping Condition
Ice Packs
Buffer
PBS, pH 7.4 with 0.02% sodium azide.
Concentration
1µg/ul
Description
Specificity: Specificity was confirmed by western blot detecting mouse superoxide dismutase (SOD1).
Format
Liquid
Purity
Filter purified
Storage
This product is stable for several weeks at 4°C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20°C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.
Storage Condition
-20C
Applications
WB
Description
A dilution of 1:500 to 1:1000 is recommended for this application. The optimal dilution should be determined by the end user., WB
Dilution
IS
Reviews of Anti-Mouse superoxide dismutase (SOD1, 24-36) Antibody