Species : |
Human |
Source : |
E.coli |
Tag : |
His |
Description : |
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. |
Form : |
Lyophilized |
Bio-activity : |
The specific activity of D2HGDH is ≥ 90,000 mU/mg. |
Molecular Mass : |
39 kDa |
AA Sequence : |
MKVLCYGVRDVELPIFEACNKEFGYDIKCVPDYLNTKETAEMAAGFDAVILRGNCFANKQNLDIYKKLGVKYILTRTAGTDHIDKEYAKELGFPMAFVPRYSPNAIAELAVTQAMMLLRHTAYTTSRTAKKNFKVDAFMFSKEVRNCTVGVVGLGRIGRVAAQIFHGMGATVIGEDVFEIKGIEDYCTQVSLDEVLEKSDIITIHAPYIKENGAVVTRDFLKKMKDGAILVNCARGQLVDTEAVIEAVESGKLGGYGCDVLDGEASVFGKDLEGQKLENPLFEKLVDLYPRVLITPHLGSYTDEAVKNMVEVSYQNLKDLAETGDCPNKIKMGSSHHHHHHSSGLVPRGSHM |
Purity : |
≥ 99% SDS-PAGE |
Applications : |
Functional Studies, SDS-PAGE |
Storage : |
Shipped at 4 centigrade. Store at +4 centigrade short term (1-2 weeks). Store at -20 centigrade. Avoid freeze/thaw cycle. This product is an active protein and may elicit a biological response in vivo, handle with caution. |
Reconstitution : |
Reconstitute in water to a concentration of 0.1-5 mg/mL. The solution can be diluted into other aqueous buffers and stored at –20 centigrade for future use. |
Full Length : |
Full L. |