|Application ||WB, IF, ICC, E|
|Other Accession||NP_001036096, 111038124|
|Calculated MW||12806 Da|
|Application Notes||SDHAF1 antibody can be used for detection of SDHAF1 by Western blot at 1 - 2 µg/mL. Antibody can also be used for immunocytochemistry starting at 5 µg/mL. For immunofluorescence start at 5 µg/mL.|
|Target/Specificity||SDHAF1; SDHAF1 antibody is predicted to not cross-react with other SDHAF protein family members.|
|Reconstitution & Storage||SDHAF1 antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.|
|Precautions||SDHAF1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||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.|
|Cellular Location||Mitochondrion matrix|
|Tissue Location||Ubiquitously expressed.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Ghezzi D, Goffrini P, Uziel G, et al. SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nat. Genet. 2009; 41:654-6.
Hensen EE and Bayley JP. Recent advances in the genetics of SDH-related paraganglioma and pheochromocytoma. Fam. Cancer 2011; 10:355-63.
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