Anti-SDHAF1 Antibody

SDHAF1 (Succinate dehydrogenase complex assembly factor 1) is one of the subunits of the succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain. SDHAF1 resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency) . Unlike the related protein SDHAF2, SDHAF1 is not thought to be a tumor suppressor.

SDHAF1 antibody was raised against an 18 amino acid synthetic peptide near the carboxy terminus of human SDHAF1. The immunogen is located within the last 50 amino acids of SDHAF1.

Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3.

SDHAF1 antibody can be stored at 4°C for three months and -20°C, stable for up to one year. Avoid repeated freeze-thaw cycles. Antibodies should not be exposed to prolonged high temperatures.