Anti-OGDH (2-oxoglutarate Dehydrogenase, Mitochondrial, 2-oxoglutarate Dehydrogenase Complex Component E1, OGDC-E1, Alpha-ketoglutarate Dehydrogenase, OGDH) Polyclonal Antibody

The alpha-ketoglutarate dehydrogenase (alpha-KGD) complex is a multienzyme complex which localizes to the mitochondrial matrix and consists of three protein subunits: alpha-ketoglutarate dehydrogenase, also designated alpha-KGD, E1k or oxoglutarate dehydrogenase (OGDH); dihydrolipoyl succinyltransferase (E2k|or DLST); and dihydrolipoyl dehydrogenase (E3). The alpha-KGD subunit of the alpha-KGD complex catalyzes the conversion of alpha-ketoglutarate to succinyl-CoA and CO2, an essential reaction of the tricarboxylic acid cycle. A definciency in alpha-KGD results in hypotonia, metabolic acidosis, hyperlactatemia immediately after birth, and neurologic deterioration resulting in death at about 30 months of age. Low molar ratios of ketone bodies in plasma of neonates with congenital lactic acidosis are proposed indicators of tricarboxylic acid cycle dysfunction.||Applications: |Suitable for use in Western Blot, Immunohistochemistry. Other applications not tested.||Recommended Dilution:|Western Blot: 1:500-1:2000|Immunohistochemistry: 1:50-1:200|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.