D2HGDH (D-2-Hydroxyglutarate Dehydrogenase, Mitochondrial)

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£614.00

SKU:: 478989
Anwendung:: WB
Reinheit:: Purified by affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to the C-terminal region of Human D2HGDH
Format:: Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Weitere Details:: This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.