Recombinant Human SDHA protein (His Tag) (PDEH100987)

For research use only.
Synonyms | SDHA, Flavoprotein subunit of complex II (Fp), SDH2, SDHF, Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial |
Species | Human |
Expression Host | E.coli |
Sequence | Ala43-Tyr664 |
Accession | P31040 |
Calculated Molecular Weight | 68.3 kDa |
Observed Molecular Weight | 70 kDa |
Tag | N-His & C-His |
Bio-activity | Not validated for activity |
Purity | > 95% as determined by reducing SDS-PAGE. |
Endotoxin | < 10 EU/mg of the protein as determined by the LAL method |
Storage | Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months. |
Shipping | This product is provided as lyophilized powder which is shipped with ice packs. |
Formulation | Lyophilized from a 0.2 μm filtered solution in PBS with 5% Trehalose and 5% Mannitol. |
Reconstitution | It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis. |
Background | Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone.Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D). A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. |
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