ACADS (Short-Chain specific acyl-CoA Dehydrogenase, Mitochondrial, SCAD, Butyryl-CoA Dehydrogenase)

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£460.00

SKU:: 304265
Reinheit:: Purified by immunoaffinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Fusion protein of human ACADS
Format:: Supplied as a liquid in PBS, pH 7.4, 0.05% sodium azide, 40% glycerol.
Weitere Details:: This gene encodes a a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. Applications: Suitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:1000-1:2000 Western Blot: 1:200-1:1000 Immunohistochemistry: 1:25-1:100 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.