The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 18 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron.
Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
Involvement in disease
Defects in ISCU are the cause of myopathy with exercise intolerance Swedish type (MEIS) [MIM:255125]; also known as myopathy with deficiency of succinate dehydrogenase and aconitase or myoglobinuria due to abnormal glycolysis or hereditary myopathy with lactic acidosis (HML). This autosomal recessive metabolic disease is characterized by lifelong severe exercise intolerance, in which minor exertion causes fatigue of active muscles, shortness of breath, and cardiac palpitations in association with lactic acidosis. The biochemical phenotype is characterized by a deficiency in mitochondrial iron-sulfur proteins and impaired muscle oxidative metabolism.