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Recombinant Human SDHA protein (His tag)
SKU: PDEH100333-100
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Recombinant Human SDHA protein (His tag)
| SKU # | PDEH100333 |
| Expression Host | E. coli |
Description
| Synonyms | Succinate dehydrogenase [ubiquinone] flavoprotein subunit;mitochondrial; SDHA;Flavoprotein subunit of complex II (Fp);SDHF;SDH2 |
| Species | Human |
| Expression_host | E.coli |
| Sequence | Ala 43-Tyr 664 |
| Accession | P31040 |
| Mol_Mass | 68.3 kDa |
| AP_Mol_Mass | 70 kDa |
| Tag | N-His & C-His |
| Bio_Activity | Not validated for activity |
Propertie
| Purity | > 95 % as determined by reducing SDS-PAGE. |
| Endotoxin level | Please contact us for more information. |
| 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 sterile PBS, pH 7.4. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the printed manual. |
| 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.