Succinate dehydrogenase [ubiquinone] flavoprotein subunit Recombinant Protein | SDHA recombinant protein
Recombinant Human Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014]
Uniprot Description
SDHA: is the catalytic subunit of succinate dehydrogenase (SDH) complex II of the mitochondrial electron transport chain. Complex II contains four subunits: the flavoprotein catalytic subunit SDHA, iron-sulfur protein SDHB, and a cytochrome b560 composed of SDHC and SDHD. Interaction with SDH5 is required for FAD attachment. Responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHA cause defective mitochondrial oxidative phosphorylation, giving rise to heterogeneous clinical symptoms ranging from isolated organ dysfunction to multisystem disorder. Acetylation of SDHA may control entry of the substrate into the active site, thus regulating its enzymatic activity. Acetylated SDHA may be a SIRT3 substrate SIRT3 is a mitochondrial NAD(+)-dependent deacetylase. Increased succinate levels as a consequence of SDH deficiency inhibit hypoxia inducible factor-1alpha (HIF-1alpha) prolyl hydroxylases leading to sustained HIF-1alpha expression in tumours. Defects in SDHA cause of Leigh syndrome, a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.3.5.1; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase; Tumor suppressor
Chromosomal Location of Human Ortholog: 5p15.33
Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion; nucleolus
Molecular Function: electron carrier activity; protein binding; succinate dehydrogenase (ubiquinone) activity; succinate dehydrogenase activity
Biological Process: anaerobic respiration; mitochondrial electron transport, succinate to ubiquinone; nervous system development; succinate metabolic process; tricarboxylic acid cycle
Disease: Cardiomyopathy, Dilated, 1gg; Leigh Syndrome; Mitochondrial Complex Ii Deficiency; Paragangliomas 5
Research Articles on SDHA
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Product Notes
The SDHA sdha (Catalog #AAA9421595) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is Partial of the Full Length of 44-664aa, 44-293aa. The Recombinant Human Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial reacts with Human and may cross-react with other species as described in the data sheet. It is sometimes possible for the material contained within the vial of "Succinate dehydrogenase [ubiquinone] flavoprotein subunit, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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