Mouse 2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial (BCKDHA) ELISA Kit-Sandwich

In conjunction with BCKDHB, the mitochondrial 2-oxoisovalerate dehydrogenase subunit alpha constitutes the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. This complex facilitates the multi-step oxidative decarboxylation of alpha-ketoacids derived from branched-chain amino acids such as valine, leucine, and isoleucine, producing CO2 and acyl-CoA. The resultant acyl-CoA is subsequently used for energy production. Bckdha is highly expressed in the mouse liver and plays a crucial role in maintaining blood glucose balance and synthesizing fatty acids. For instance, the absence of Bckdha results in diminished fatty acid synthesis and significantly impacts fatty acid diversity. Furthermore, Bckdha deficiency interferes with glucose metabolism, leading to decreased glucose uptake and a lowered glycolytic flux.