alpha-KGD Anticorpi

The å-ketoglutarate dehydrogenase (å-KGD) complex is a multienzyme complex which localizes to the mitochondrial matrix and consists of three protein subunits: å-ketoglutarate dehydrogenase, also designated å-KGD, E1k or oxoglutarate dehydrogenase (OGDH); dihydrolipoyl succinyltransferase (E2k or DLST); and dihydrolipoyl dehydrogenase (E3). The å-KGD subunit of the å-KGD complex catalyzes the conversion of å-ketoglutarate to succinyl-CoA and CO2, an essential reaction of the tricarboxylic acid cycle. A definciency in å-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.