In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol.
Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB) and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane.
Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders.
Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations associate with juvenile encephalopathy as well as Leigh syndrome, a severe neurological disorder.
Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal 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.
仕様
Size:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity:Recognizes endogenous levels of SDHB. Species Crossreactivity: Human, mouse, rat