Anti-SDHA Antibody [SDHA/7491] (A315627)

Anti-SDHA Antibody [SDHA/7491]

Mouse monoclonal [SDHA/7491] antibody to SDHA.

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 are associated 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.

Expressed strongly in small intestine. Also expressed in prostate, colon, stomach and salivary gland.

IHC: 1-2 µg/ml

Human

Recombinant fragment (around Amino Acids 450-650) of human SDHA protein. The exact sequence is proprietary.

Mouse

Monoclonal

IgG2

kappa

Protein A/G chromatography.

200 µg/ml

70 kDa

Liquid

Supplied in 10mM Phosphate Buffered Saline with 0.05% BSA and 0.05% Sodium Azide.

Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Flavoprotein subunit of complex II, Fp, Malate dehydrogenase [quinone] flavoprotein subunit, SDH2, SDHF, Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial