Recombinant Human SDHA Protein, N-His (重组蛋白) | Bioss

bs-103320P

Recombinant Human SDHA Protein, N-His

SDH2; Flavoprotein subunit of complex II; Succinate dehydrogenase [ubiquinone] flavoprotein subunit; mitochondrial; SDHA; SDHF; Fp

Lyophilized

AC

70.31 kDa

Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.

Not tested

44-664/664

Human

N-His

>90% as determined by SDS-PAGE.

E.coli

Reconstitute in sterile water for a stock solution.

Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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.