Mouse 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) ELISA Kit-Sandwich

The mitochondrial 2-oxoisovalerate dehydrogenase subunit beta, in combination with BCKDHA, assembles the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. This complex is responsible for catalyzing the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino acids valine, leucine, and isoleucine, leading to the production of CO2 and acyl-CoA, which is subsequently utilized for energy production. Bckdhb knockout mice (Bckdhb−/−) have been used to investigate the pathophysiology of classical diabetes known as Maple Syrup Urine Disease (MSUD). MSUD is a rare metabolic disorder caused by a defect in the branched-chain α-ketoacid dehydrogenase complex (BCKDH), resulting in the buildup of branched-chain amino acids such as leucine, isoleucine, and valine. Bckdhb−/− mice exhibit severe early-onset symptoms akin to human MSUD, including developmental delays, feeding challenges, neurological abnormalities, and early mortality.